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1. Effectiveness and safety of tocilizumab in patients with systemic sclerosis: a propensity score matched controlled observational study of the EUSTAR cohort

Author: Pavel I Novikov, Thierry Martin, Eric Hachulla, Vanessa Smith, Maurizio Cutolo, Yannick Allanore, Camillo Ribi, Oliver Distler, Britta Maurer, Paolo Airo, Cosimo Bruni, Veronica Codullo, Luc Mouthon, Marie Vanthuyne, Elisabetta Zanatta, Simona Rednic, Ulf Müller-Ladner, Nemanja Damjanov, Anna-Maria Hoffmann-Vold, Michele Iudici, Suzana Jordan, Jean Sibilia, Mengtao Li, Roger Hesselstrand, Elise Siegert, László Czirják, Armando Gabrielli, Dominique Farge Bancel, Yolanda Braun-Moscovici, Nicolas Hunzelmann, Raffaele Pellerito, Jörg Henes, Bojana Stamenkovic, Carlo Francesco Selmi, Mohammed Tikly, Merete Engelhart, Valeria Riccieri, Cord Sunderkötter, Francesca Ingegnoli, Francesco Paolo Cantatore, Susanne Ullman, Maria Rosa Pozzi, Piotr Wiland, Juan Jose Alegre-Sancho, Brigitte Krummel-Lorenz, Ellen De Langhe, Branimir Anic, Carolina de Souza Müller, Simon Stebbings, Alessandra Vacca, Lisa Stamp, Kamal Solanki, Douglas Veale, Esthela Loyo, Edoardo Rosato, Cristina-Mihaela Tanaseanu, Rosario Foti, Codrina Ancuta, Ira Litinsky, Lesley Ann Saketkoo, Eduardo Kerzberg, Tim Schmeiser, Piercarlo Sarzi Puttini, Fabio Cacciapaglia, Marie-Elise Truchetet, Muriel Elhai, Juan José Alegre Sancho, Ivan Castellví, Serena Vettori, Vivien M Hsu, Masataka Kuwana, Sule Yavuz, Radim Becvar, Patricia E Carreira, Eugene J Kucharz, Bernard Coleiro, Paul Hasler, Carlos de la Puente, Svetlana Agachi, Thierry Zenone, Francesco Del Galdo, Doron Rimar, Alessandro Giollo, Lidia P Ananieva, Ina Kötter, Mislav Radic, Dominik Majewski, Kristine Herrmann, Goda Seskute, Lorinda S Chung, Laura Belloli, Ulrike Held, FJ López-Longo, Joerg H W Distler, Maria João Salvador, Simon Kuster, Klaus Steigmiller, Ana-Maria Gheorghiu, Katarzyna Romanowska-Prochnicka, Yuichiro Shirai, Chris Denton, Ruxandra M Ionescu, Stefan Heitmann Vera Ortiz-Santamaria, Walid Ahmed Abdel Atty Mohamed, Paloma Garcíadela Peña Lefebvre, and Vera Bernardino
Subjects: Medicine
Abstract: Objectives Tocilizumab showed trends for improving skin fibrosis and prevented progression of lung fibrosis in systemic sclerosis (SSc) in randomised controlled clinical trials. We aimed to assess safety and effectiveness of tocilizumab in a real-life setting using the European Scleroderma Trial and Research (EUSTAR) database.Methods Patients with SSc fulfilling the American College of Rheumatology (ACR)/EULAR 2013 classification criteria, with baseline and follow-up visits at 12±3 months, receiving tocilizumab or standard of care as the control group, were selected. Propensity score matching was applied. Primary endpoints were the modified Rodnan skin score (mRSS) and FVC at 12±3 months compared between the groups. Secondary endpoints were the percentage of progressive/regressive patients for skin and lung at 12±3 months.Results Ninety-three patients with SSc treated with tocilizumab and 3180 patients with SSc with standard of care fulfilled the inclusion criteria. Comparison between groups did not show significant differences, but favoured tocilizumab across all predefined primary and secondary endpoints: mRSS was lower in the tocilizumab group (difference −1.0, 95% CI −3.7 to 1.8, p=0.48). Similarly, FVC % predicted was higher in the tocilizumab group (difference 1.5 (−6.1 to 9.1), p=0.70). The percentage of progressive/regressive patients favoured tocilizumab over controls. These results were robust regarding the sensitivity analyses. Safety analysis confirmed previously reported adverse event profiles.Conclusion Although this large, observational, controlled, real-life EUSTAR study did not show significant effectiveness of tocilizumab on skin and lung fibrosis, the consistency of direction of all predefined endpoints generates hypothesis for potential effectiveness in a broader SSc population.
Published: 2022
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2. Presentation and Real-World Management of Giant Cell Arteritis (Artemis Study)

Author: Alfred Mahr, Eric Hachulla, Hubert de Boysson, Nassim Guerroui, Emmanuel Héron, Stéphane Vinzio, Jonathan Broner, François-Xavier Lapébie, Martin Michaud, Laurent Sailler, Thierry Zenone, Mohamed Djerad, Mathieu Jouvray, Emilie Shipley, Nathalie Tieulie, Guillaume Armengol, Bastien Bouldoires, Jean-Francois Viallard, Isabelle Idier, Marc Paccalin, and Valérie Devauchelle-Pensec
Subjects: giant cell arteritis, phenotype [mesh], management - healthcare, observational, glucocorticoids (GCs), methotrexate, Medicine (General), R5-920
Abstract: Background: Few studies of daily practice for patients with giant cell arteritis (GCA) are available. This French study aimed to describe the characteristics and management of GCA in a real-life setting.Methods: Cross-sectional, non-interventional, multicenter study of patients ≥50 years old who consulted hospital-based specialists for GCA and were under treatment. Patient characteristics and journey, diagnostic methods and treatments were collected. Descriptive analyses were performed.Results: In total, 306 patients (67% females, mean age 74 ± 8 years old) were recruited by 69 physicians (internists: 85%, rheumatologists: 15%); 13% of patients had newly diagnosed GCA (diagnosis-to-visit interval
Published: 2021
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3. Cornea verticillata and acroparesthesia efficiently discriminate clusters of severity in Fabry disease.

Author: Wladimir Mauhin, Olivier Benveniste, Damien Amelin, Clémence Montagner, Foudil Lamari, Catherine Caillaud, Claire Douillard, Bertrand Dussol, Vanessa Leguy-Seguin, Pauline D'Halluin, Esther Noel, Thierry Zenone, Marie Matignon, François Maillot, Kim-Heang Ly, Gérard Besson, Marjolaine Willems, Fabien Labombarda, Agathe Masseau, Christian Lavigne, Didier Lacombe, Hélène Maillard, and Olivier Lidove
Subjects: Medicine, Science
Abstract: BACKGROUD:Fabry disease (OMIM #301 500), the most prevalent lysosomal storage disease, is caused by enzymatic defects in alpha-galactosidase A (GLA gene; Xq22.1). Fabry disease has historically been characterized by progressive renal failure, early stroke and hypertrophic cardiomyopathy, with a diminished life expectancy. A nonclassical phenotype has been described with an almost exclusive cardiac involvement. Specific therapies with enzyme substitution or chaperone molecules are now available depending on the mutation carried. Numerous clinical and fundamental studies have been conducted without stratifying patients by phenotype or severity, despite different prognoses and possible different pathophysiologies. We aimed to identify a simple and clinically relevant way to classify and stratify patients according to their disease severity. METHODS:Based on data from the French Fabry Biobank and Registry (FFABRY; n = 104; 54 males), we applied unsupervised multivariate statistics to determine clusters of patients and identify clinical criteria that would allow an effective classification of adult patients. Thanks to these criteria and empirical clinical considerations we secondly elaborate a new score that allow the severity stratification of patients. RESULTS:We observed that the absence of acroparesthesia or cornea verticillata is sufficient to classify males as having the nonclassical phenotype. We did not identify criteria that significantly cluster female patients. The classical phenotype was associated with a higher risk of severe renal (HR = 35.1; p
Published: 2020
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4. Deep characterization of the anti-drug antibodies developed in Fabry disease patients, a prospective analysis from the French multicenter cohort FFABRY

Author: Wladimir Mauhin, Olivier Lidove, Damien Amelin, Foudil Lamari, Catherine Caillaud, Federico Mingozzi, Gaëlle Dzangué-Tchoupou, Louiza Arouche-Delaperche, Claire Douillard, Bertrand Dussol, Vanessa Leguy-Seguin, Pauline D’Halluin, Esther Noel, Thierry Zenone, Marie Matignon, François Maillot, Kim-Heang Ly, Gérard Besson, Marjolaine Willems, Fabien Labombarda, Agathe Masseau, Christian Lavigne, Roseline Froissart, Didier Lacombe, Jean Marc Ziza, Eric Hachulla, and Olivier Benveniste
Subjects: Fabry disease, Anti-drug antibodies, Agalsidase, Lysosomal storage disease, Enzyme replacement therapy, IgG4, Medicine
Abstract: Abstract Background Fabry disease (OMIM #301500) is an X-linked disorder caused by alpha-galactosidase A deficiency with two major clinical phenotypes: classic and non-classic of different prognosis. From 2001, enzyme replacement therapies (ERT) have been available. We aimed to determine the epidemiology and the functional characteristics of anti-drug antibodies. Patients from the French multicenter cohort FFABRY (n = 103 patients, 53 males) were prospectively screened for total anti-agalsidase IgG and IgG subclasses with a home-made enzyme-linked immunosorbent assay (ELISA), enzyme-inhibition assessed with neutralization assays and lysoGb3 plasma levels, and compared for clinical outcomes. Results Among the patients exposed to agalsidase, 40% of men (n = 18/45) and 8% of women (n = 2/25) had antibodies with a complete cross-reactivity towards both ERTs. Antibodies developed preferentially in men with non-missense GLA mutations (relative risk 2.88, p = 0.006) and classic phenotype (58.6% (17/29) vs 6.7% (1/16), p = 0.0005). Specific anti-agalsidase IgG1 were the most frequently observed (16/18 men), but the highest concentrations were observed for IgG4 (median 1.89 μg/ml, interquartile range (IQR) [0.41–12.24]). In the men exposed to agalsidase, inhibition was correlated with the total IgG titer (r = 0.67, p
Published: 2018
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5. Systemic Bartonella henselae Infection in Immunocompetent Adult Presenting as Fever of Unknown Origin

Author: Thierry Zenone
Subjects: Medicine
Abstract: Systemic clinical presentations of infection caused by Bartonella henselae are rare in immunocompetent adults. We report four cases with hepatic and/or splenic involvement, presenting as fever of unknown origin. We discuss diagnosis and treatment of this infection. Bartonella henselae serology allows an easy diagnosis of hepatosplenic involvement in cat scratch disease, a clinical picture that appears to be underrecognized.
Published: 2011
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6. An international SUrvey on non-iNvaSive tecHniques to assess the mIcrocirculation in patients with RayNaud’s phEnomenon (SUNSHINE survey)

Author: Ingegnoli, Francesca, Ughi, Nicola, Dinsdale, Graham, Orenti, Annalisa, Boracchi, Patrizia, Allanore, Yannick, Foeldvari, Ivan, Sulli, Alberto, Cutolo, Maurizio, Smith, Vanessa, Herrick, Ariane L., Hij, Adrian, Sulli, Alberto, Nitsche, Alejandro, Vacca, Alessandra, Balbir-Gurman, Alexandra, Abdessemed, Amina, Vargas, Angelica, Valenzuela, Antonia, Makol, Ashima, Baranauskaite, Asta, Derfalvi, Beata, Serrano Benavente, Belén, Sozeri, Betul, Bica, Blanca E., Stamenkovic, Bojana, Mihai, Carina, Chizzolini, Carlo, Abud Mendoza, Carlos, de la Puente, Carlos, von Muhlen, Carlos, Bertolazzi, Chiara, Pain, Clare, Ickinger, Claudia, Ancuta, Codrina, Sunderkotter, Cord, Kayser, Cristiane, De Araujo, Daniel B., Launay, David, Khanna, Dinesh, Krasowska, Dorota, Veale, Douglas, Kaliterna, Dušanka M., Rosato, Edoardo, de Langhe, Ellen, Hachulla, Eric, Naredo, Esperanza, Loyo, Esthela, Alvarez Hernández, Everardo, Sztajnbok, Flavio, Boin, Francesco, Longo, Francisco J., van den Hoogen, Frank, Hernandez Molina, Gabriela, Riemekasten, Gabriela, Szucs, Gabriella, Moroncini, Gianluca, Fragoso Loyo, Hilda, Dobrev, Hristo, Janta, Iustina, Cracowski, Jean-Luc, Pauling, John, Akikusa, Jonathan, Sotoca Fernàndez, Jorge, Khan Ajaz, Kariem, Solanki, Kamal, Wierzba, Karol, Romanowska Próchnicka, Katarzyna, Rouster Stevens, Kelly, Belloli, Laura, Lewandowski, Laura, Santos, Lelita, Saketkoo, Lesley A., Ananyeva, Lidia, Beretta, Lorenzo, Michalska Jakubus, Małgorzata, Audisio, Marcelo J., Milchert, Marcin, Molina, Maria J., Moraes, Fontes Maria F., Terreri, Maria T., Puszczewicz, Mariusz, Barešić, Marko, Hufnagel, Markus, Mamani, Marta N., Gutierrez, Marwin, Curran, Megan, Hughes, Michael, Becker, Mike, Inanç, Murat, Petraitis, Mykolas, Juan Carlos, Nieto-Gonzàlez, Fathi, Nihal, Aktay Ayaz, Nuray, Distler, Oliver, Sander, Oliver, Ömer, Pamuk N., García dela Peña Lefebvre, Paloma, Caramaschi, Paola, Hasler, Paul, Ostojic, Predrag, Bečvář, Radim, Rodríguez, Reyna S. Tatiana, Lima, Rodrigo, Hesselstrand, Roger, Cimaz, Rolando, Irace, Rosaria, Petty, Ross, de Angelis, Rossella, Dobrota, Rucsandra, Payne-Poff, Sarah, Kubo, Satoshi, Guiducci, Serena, Popa, Serghei, Lambova, Sevdalina, Stebbings, Simon, Rednic, Simona, Yavuz, Sule, Benseler, Susa, Shevtsova, Tatzana, Daikeler, Thomas, Schmeiser, Tim, Frech, Tracy, Minier, Tünde, Müller Ladner, Ulf, Walker, Ulrich, Riccieri, Valeria, Vilela, Verônica, Hermann, Walter, Braun-Moscovici, Yolanda, Uziel, Yosef, Thierry, Zenone, and On behalf of the EULAR Study Group on Microcirculation in RheumaticDiseases
Published: 2017
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7. Syndrome de Cogan

Author: J. Pouchot, Arsène Mekinian, Olivier Fain, and Thierry Zenone
Subjects: Gynecology, medicine.medical_specialty, business.industry, Gastroenterology, Internal Medicine, medicine, business
Abstract: Resume Le syndrome de Cogan « typique » est defini par une keratite interstitielle non-syphilitique associee a une atteinte audio-vestibulaire proche du syndrome de Meniere avec un delai maximum de 2 ans entre les atteintes. Le syndrome de Cogan est dit « atypique » en presence d'une atteinte oculaire inflammatoire autre que la keratite interstitielle, d'un tableau audio-vestibulaire different de celui evoquant un syndrome de Meniere, ou d'un delai de plus de 2 ans entre les atteintes ophtalmologiques et audio-vestibulaires. D'autres manifestations sont possibles, principalement des signes generaux et une vascularite le plus souvent des gros vaisseaux touchant volontiers l'aorte thoracique. La presence d'un syndrome inflammatoire est habituelle, et aucun test biologique specifique n'est disponible. Le pronostic est domine par l'atteinte audio-vestibulaire et en particulier le risque de surdite definitive, d'autres complications en particulier les complications vasculaires etant rares. Le traitement fait appel a la corticotherapie et le recours aux autres immunosuppresseurs ou aux biotherapies reste mal codifie.
Published: 2021

8. Systematic retrospective study of 64 patients with anti-Mi2 dermatomyositis: A classic skin rash with a necrotizing myositis and high risk of malignancy

Author: Sarah Guégan, Nathalie Costedoat-Chalumeau, Vincent Descamps, Emmanuel Bergot, G. Monseau, Yoland Schoindre, C. Martel, Stéphane Barete, Cristian Bulai Livideanu, Valérie Jooste, Aude Rigolet, Achille Aouba, Thierry Zenone, Hubert de Boysson, Emmanuelle Salort-Campana, Jean-Emmanuel Kahn, Sarah Leonard-Louis, Werner Stenzel, Baptiste Hervier, Sylvain Audia, Antoine Dossier, Nicolas Schleinitz, Eric Hachulla, Alain Meyer, Laurent Mortier, Marie Lacoste, Guillaume Moulis, Serge Madaule, Florian Perez, Kuberaka Mariampillai, Anne-Marie Bouvier, Yves Allenbach, Laurence Verneuil, Benjamin Terrier, Nicolas Champtiaux, Thomas Papo, Séverine Genot, Maxime Samson, Agathe Masseau, Thierry Maisonobe, Marie-Aleth Richard, Olivier Benveniste, Boris Bienvenu, François Maurier, Guillaume Lefèvre, Anne-Marie Piette, and O. Landon-Cardinal
Subjects: Adult, Male, medicine.medical_specialty, Risk of malignancy, MEDLINE, Dermatology, Risk Assessment, Dermatomyositis, Necrosis, Risk Factors, Neoplasms, medicine, Humans, Muscle, Skeletal, Aged, Autoantibodies, Retrospective Studies, Skin, business.industry, Incidence, Necrotizing myositis, Retrospective cohort study, Exanthema, Middle Aged, Prognosis, medicine.disease, Rash, Female, medicine.symptom, business, Mi-2 Nucleosome Remodeling and Deacetylase Complex
Published: 2020

9. Presentation and Real-World Management of Giant Cell Arteritis (Artemis Study)

Author: Valérie Devauchelle-Pensec, Hubert de Boysson, Nathalie Tieulie, Thierry Zenone, Mathieu Jouvray, Nassim Guerroui, Guillaume Armengol, Martin Michaud, Emmanuel Héron, Emilie Shipley, Jean-François Viallard, Eric Hachulla, Marc Paccalin, Bastien Bouldoires, Mohamed Djerad, Stéphane Vinzio, Laurent Sailler, François-Xavier Lapébie, Jonathan Broner, Alfred Mahr, and Isabelle Idier
Subjects: medicine.medical_specialty, Medicine (General), glucocorticoids (GCs), Constitutional symptoms, management - healthcare, methotrexate, Polymyalgia rheumatica, chemistry.chemical_compound, tocilizumab, Tocilizumab, R5-920, Interquartile range, Internal medicine, Diabetes mellitus, medicine, observational, skin and connective tissue diseases, Original Research, business.industry, giant cell arteritis, General Medicine, phenotype [mesh], medicine.disease, Comorbidity, Giant cell arteritis, chemistry, Medicine, business, Dyslipidemia
Abstract: Background: Few studies of daily practice for patients with giant cell arteritis (GCA) are available. This French study aimed to describe the characteristics and management of GCA in a real-life setting.Methods: Cross-sectional, non-interventional, multicenter study of patients ≥50 years old who consulted hospital-based specialists for GCA and were under treatment. Patient characteristics and journey, diagnostic methods and treatments were collected. Descriptive analyses were performed.Results: In total, 306 patients (67% females, mean age 74 ± 8 years old) were recruited by 69 physicians (internists: 85%, rheumatologists: 15%); 13% of patients had newly diagnosed GCA (diagnosis-to-visit interval 18FDG-PET, and aortic angio-CT, respectively. All patients received glucocorticoids, which were ongoing for 89%; 29% also received adjunct medication(s) (methotrexate: 19%, tocilizumab: 15%). A total of 40% had relapse(s); the median time to the first relapse was 10 months. Also, 37% had comorbidity(ies) related to or aggravated by glucocorticoids therapy.Conclusion: This large observational study provides insight into current medical practices for GCA. More than one third of patients had comorbidities related to glucocorticoid therapy for a median disease duration of 13 months. Methotrexate and tocilizumab were the most common adjunct medications.
Published: 2021

10. Clinical value of a [18F]-FDG PET-CT muscle-to-muscle SUV ratio for the diagnosis of active dermatomyositis

Author: Anne-Sophie Morin, Aurélie Grados, Benoit Brihaye, Éric Denis, Olivier Lidove, Thierry Zenone, Yoland Schoindre, Fanny Andry, Benjamin Terrier, Alexandra Audemard, Nihal Martis, Emeline Castela, Denis Quinsat, Mikael Ebbo, Nicolas Mounier, Stéphane Liguori, Laurent Marcq, Philippe Viau, and Jean-Philippe Chaborel
Subjects: Adult, Male, medicine.medical_specialty, Sensitivity and Specificity, Dermatomyositis, 030218 nuclear medicine & medical imaging, 03 medical and health sciences, Visual grading, 0302 clinical medicine, Fluorodeoxyglucose F18, Positron Emission Tomography Computed Tomography, medicine, Humans, Radiology, Nuclear Medicine and imaging, Muscle, Skeletal, Aged, Retrospective Studies, Aged, 80 and over, Muscle biopsy, medicine.diagnostic_test, business.industry, Ultrasound, Reproducibility of Results, General Medicine, Middle Aged, medicine.disease, Control subjects, Positron emission tomography, 030220 oncology & carcinogenesis, Clinical value, Female, Fdg pet ct, Radiology, Radiopharmaceuticals, business, Nuclear medicine
Abstract: To study a muscle-to-muscle standardised uptake value (SUV) ratio with FDG-PET/CT (FDG-PET) as a marker for the detection of disease activity in dermatomyositis (DM). Patients with DM (n = 24) who met the European Neuro-Muscular Centre diagnostic criteria were retrospectively identified over a 3-year period through a national survey. Muscle biopsy was performed in all patients. Maximum SUV was measured in proximal muscles (SUVPROX) that had the highest radiotracer uptake on visual grading as well as in the musculus longissimus thoracis (SUVMLT), whereas mean SUV was measured for the liver (SUVLIV). Muscle-to-liver SUV ratios for either muscle group were compared and a SUVPROX/SUVMLT ratio was calculated. SUVPROX/SUVMLT of DM patients were compared with age- and sex-matched control subjects (n = 24) with melanoma who had received FDG-PET scans. DM patients presented with proximal and symmetrical muscle uptake. Differences in SUVPROX/SUVLIV and SUVMLT/SUVLIV ratios in DM subjects were significant (p
Published: 2019

11. Cornea verticillata and acroparesthesia efficiently discriminate clusters of severity in Fabry disease

Author: Marie Matignon, Olivier Benveniste, Agathe Masseau, Kim-Heang Ly, Didier Lacombe, Gérard Besson, François Maillot, Christian Lavigne, D. Amelin, Esther Noel, Foudil Lamari, Catherine Caillaud, Wladimir Mauhin, Hélène Maillard, Olivier Lidove, C. Montagner, Thierry Zenone, Marjolaine Willems, Vanessa Leguy-Seguin, Pauline D’Halluin, Bertrand Dussol, Fabien Labombarda, Claire Douillard, Groupe Hospitalier Diaconesses Croix Saint-Simon, Centre de recherche en Myologie – U974 SU-INSERM, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Service de médecine interne [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Service de Biochimie Métabolique [CHU Pitié-Salpêtrière], CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Sorbonne Université (SU), Service de biochimie métabolique [CHU Necker], CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut Necker Enfants-Malades (INEM - UM 111 (UMR 8253 / U1151)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Paris (UP), Hôpital Jeanne de Flandres, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Aix Marseille Université (AMU), Centre d'Investigation Clinique [Hôpital de la Conception - APHM] (CIC), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Institut Paoli-Calmettes, Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hôpital de la Conception [CHU - APHM] (LA CONCEPTION), Assistance Publique - Hôpitaux de Marseille (APHM), Service de médecine interne et immunologie clinique (SOC 1) [CHU de Dijon], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Le CHCB, Centre Hospitalier de la Côte Basque, CHU Strasbourg, Centre hospitalier de Valence, Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-IFR10, Groupe Henri Mondor-Albert Chenevier, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Hôpital Albert Chenevier, Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Imagerie et cerveau (iBrain - Inserm U1253 - UNIV Tours ), Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire [Grenoble] (CHU), CHU Montpellier, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Service de médecine interne [Nantes], Université de Nantes (UN)-Hôtel-Dieu-Centre hospitalier universitaire de Nantes (CHU Nantes), Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), CHU Bordeaux [Bordeaux], Laboratoire Maladies Rares: Génétique et Métabolisme (Bordeaux) (U1211 INSERM/MRGM), Université de Bordeaux (UB)-Groupe hospitalier Pellegrin-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital Claude Huriez [Lille], CHU Lille, Service de Département de médecine interne et immunologie clinique [CHU Pitié-Salpêtrière] (DMIIC), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPCité), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Centre de Recherche en Myologie, Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), Université de Tours-Institut National de la Santé et de la Recherche Médicale (INSERM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), and Centre Hospitalier de la Côte Basque (CHCB)
Subjects: Hydrolases, 030232 urology & nephrology, Otology, 030204 cardiovascular system & hematology, Deafness, Pathology and Laboratory Medicine, Vascular Medicine, Biochemistry, Cornea, Cohort Studies, 0302 clinical medicine, Lysosomal storage disease, Medicine and Health Sciences, Renal Transplantation, Cornea verticillata, Prospective Studies, Registries, Hearing Disorders, Multidisciplinary, Proteinuria, Hypertrophic cardiomyopathy, Middle Aged, [SDV.MHEP.EM]Life Sciences [q-bio]/Human health and pathology/Endocrinology and metabolism, Phenotype, 3. Good health, Enzymes, Stroke, Neurology, Medicine, Female, France, medicine.symptom, Anatomy, Cardiomyopathies, Research Article, Adult, medicine.medical_specialty, Glycoside Hydrolases, Science, Ocular Anatomy, Cerebrovascular Diseases, Cardiology, Surgical and Invasive Medical Procedures, Urinary System Procedures, 03 medical and health sciences, Young Adult, Signs and Symptoms, Ocular System, Diagnostic Medicine, Internal medicine, medicine, Genetics, Humans, Paresthesia, Acroparesthesia, Transplantation, business.industry, Biology and Life Sciences, Proteins, Human Genetics, Organ Transplantation, medicine.disease, Fabry disease, Human genetics, Otorhinolaryngology, Enzymology, Fabry Disease, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, business
Abstract: International audience; Backgroud: Fabry disease (OMIM #301 500), the most prevalent lysosomal storage disease, is caused by enzymatic defects in alpha-galactosidase A (GLA gene; Xq22.1). Fabry disease has historically been characterized by progressive renal failure, early stroke and hypertrophic cardiomyopathy, with a diminished life expectancy. A nonclassical phenotype has been described with an almost exclusive cardiac involvement. Specific therapies with enzyme substitution or chaperone molecules are now available depending on the mutation carried. Numerous clinical and fundamental studies have been conducted without stratifying patients by phenotype or severity, despite different prognoses and possible different pathophysiologies. We aimed to identify a simple and clinically relevant way to classify and stratify patients according to their disease severity.Methods: Based on data from the French Fabry Biobank and Registry (FFABRY; n = 104; 54 males), we applied unsupervised multivariate statistics to determine clusters of patients and identify clinical criteria that would allow an effective classification of adult patients. Thanks to these criteria and empirical clinical considerations we secondly elaborate a new score that allow the severity stratification of patients.Results: We observed that the absence of acroparesthesia or cornea verticillata is sufficient to classify males as having the nonclassical phenotype. We did not identify criteria that significantly cluster female patients. The classical phenotype was associated with a higher risk of severe renal (HR = 35.1; p
Published: 2020

12. Significant weight loss in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database

Author: Michael Hughes, Calvin Heal, Elise Siegert, Eric Hachulla, Paolo Airó, Antonella Riccardi, Oliver Distler, Marco Matucci-Cerinic, Andrea Doria, Lorenzo Baretta, Alexandra Balbir-Gurman, Patricia E Carreira, Vanessa Smith, Carlos Alberto, Jörg Distler von Mühlen, Ulf Müller-Ladner, Lidia P Ananieva, László Czirják, Jörg Henes, Jeska de Vries-Bouwstra, Mengtao Li, Fabian Mendoza, Nemanja Damjanov, Ivan Castellví, Alessandro Giollo, Stefan Heitmann, Edoardo Rosato, Lorenzo Dagna, Christopher P Denton, Marie Vanthuyne, Fabio Cacciapaglia, Valeria Riccieri, Nicolas Hunzelmann, Ami Shah, Carlomaurizio Montecucco, Raffaele Pellerito, Ruxandra Maria Ionescu, Simona Rednic, Ulrich Walker, Maria Rosa Pozzi, Anna-Maria Hoffmann-Vold, Marie-Elise Truchetet, Susanne Ullman, Carolina de Souza Müller, Juan Jose Alegre-Sancho, Eduardo Kerzberg, Francesco Del Galdo, Gabriela Riemekasten, Branimir Anic, Marko Baresic, Miroslav Mayer, Fahrettin Oksel, Figen Yargucu, Ellen De Langhe, Ina Kötter, Mohammed Tikly, Radim Becvar, Douglas Veale, Dorota Krasowska, Andrea Lo Monaco, Lidia Rudnicka, Ana Maria Gheorghiu, Piercarlo Sarzi Puttini, Mislav Radic, Armando Gabrielli, Maria João Salvador, Carlos de la Puente, Gabriela Szücs, Sule Yavuz, Rosario Foti, Otylia Kowal Bielecka, Codrina Ancuta, Peter Villiger, Sabine Adler, Patrick Jego, Michaela Kohm, Eugene J Kucharz, Dominique Farge Bancel, Tim Schmeiser, Alberto Cauli, Alessandra Vacca, Kamal Solanki, Piotr Wiland, Paloma García de la Peña Lefebvre, Jorge Juan Gonzalez Martin, Sergio Jimenez, Lesley Ann Saketkoo, Roger Hesselstrand, Francesca Ingegnoli, Jean Sibilia, Merete Engelhart, Esthela Loyo, Carmen Tineo, Francesco Paolo Cantatore, Brigitte Krummel-Lorenz, Petros Sfikakis, Cristiane Kayser, Vera Ortiz Santamaria, Bojana Stamenkovic, Giovanna Cuomo, Francesco Puppo, Thierry Zenone, Nihal Fathi, Ira Litinsky, Carlo Chizzolini, Monika Swacha, Washington Bianchi, Breno Valdetaro Bianchi, Maria Üprus, Kati Otsa, Masataka Kuwana, Panayiotis Vlachoyiannopoulos, Sarah Kahl, Bernard Coleiro, François Spertini, Walid Ahmed Abdel Atty Mohamed, Sergey Moiseev, Pavel Novikov, Dominik Majewski, Simon Stebbings, Svetlana Agachi, Massimiliano Limonta, Carlo Francesco Selmi, Elena Rezus, Kristine Herrmann, Brigitte Granel, Goda Seskute, Matthias Seidel, Paul Hasler, Maurizio Cutolo Vera Bernardino, Carmen Pizzorni, Jadranka Morovic-Vergles, Daniel Furst, Ana-Maria Ramazan, Gianluigi Bajocchi, Lisa Stamp, Doron Rimar, Antonella Marcoccia, Srdan Novak, Luc Mouthon, Jiri Stork, Lorinda S Chung, Hadi Poormoghim, Francis Gaches, Laura Belloli, Cristina-Mihaela Tanaseanu, Fabiola Atzeni, Kilian Eyerich, Ivien M Hsu, Jacob van Laar, Mary Ellen Csuka, Omer Nuri Pamuk, Maura Couto, Arsene Mekinian, Murat Inanc, Ivan Foeldvari, Julia Martínez-Barrio, Yair Levy, Juliana Markus, Susana Oliveira, Hughes, Michael, Heal, Calvin, Siegert, Elise, Hachulla, Eric, Airó, Paolo, Riccardi, Antonella, Distler, Oliver, Matucci-Cerinic, Marco, Doria, Andrea, Baretta, Lorenzo, Balbir-Gurman, Alexandra, E Carreira, Patricia, Smith, Vanessa, Alberto, Carlo, Distler von Mühlen, Jörg, Müller-Ladner, Ulf, P Ananieva, Lidia, Czirják, László, Henes, Jörg, de Vries-Bouwstra, Jeska, Li, Mengtao, Mendoza, Fabian, Damjanov, Nemanja, Castellví, Ivan, Giollo, Alessandro, Heitmann, Stefan, Rosato, Edoardo, Dagna, Lorenzo, P Denton, Christopher, Vanthuyne, Marie, Cacciapaglia, Fabio, Riccieri, Valeria, Hunzelmann, Nicola, Shah, Ami, Montecucco, Carlomaurizio, Pellerito, Raffaele, Maria Ionescu, Ruxandra, Rednic, Simona, Walker, Ulrich, Rosa Pozzi, Maria, Hoffmann-Vold, Anna-Maria, Truchetet, Marie-Elise, Ullman, Susanne, de Souza Müller, Carolina, Jose Alegre-Sancho, Juan, Kerzberg, Eduardo, Del Galdo, Francesco, Riemekasten, Gabriela, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Oksel, Fahrettin, Yargucu, Figen, De Langhe, Ellen, Kötter, Ina, Tikly, Mohammed, Becvar, Radim, Veale, Dougla, Krasowska, Dorota, Lo Monaco, Andrea, Rudnicka, Lidia, Maria Gheorghiu, Ana, Sarzi Puttini, Piercarlo, Radic, Mislav, Gabrielli, Armando, João Salvador, Maria, de la Puente, Carlo, Szücs, Gabriela, Yavuz, Sule, Foti, Rosario, Kowal Bielecka, Otylia, Ancuta, Codrina, Villiger, Peter, Adler, Sabine, Jego, Patrick, Kohm, Michaela, J Kucharz, Eugene, Farge Bancel, Dominique, Schmeiser, Tim, Cauli, Alberto, Vacca, Alessandra, Solanki, Kamal, Wiland, Piotr, García de la Peña Lefebvre, Paloma, Juan Gonzalez Martin, Jorge, Jimenez, Sergio, Ann Saketkoo, Lesley, Hesselstrand, Roger, Ingegnoli, Francesca, Sibilia, Jean, Engelhart, Merete, Loyo, Esthela, Tineo, Carmen, Paolo Cantatore, Francesco, Krummel-Lorenz, Brigitte, Sfikakis, Petro, Kayser, Cristiane, Ortiz Santamaria, Vera, Stamenkovic, Bojana, Cuomo, Giovanna, Puppo, Francesco, Zenone, Thierry, Fathi, Nihal, Litinsky, Ira, Chizzolini, Carlo, Swacha, Monika, Bianchi, Washington, Valdetaro Bianchi, Breno, Üprus, Maria, Otsa, Kati, Kuwana, Masataka, Vlachoyiannopoulos, Panayioti, Kahl, Sarah, Coleiro, Bernard, Spertini, Françoi, Ahmed Abdel Atty Mohamed, Walid, Moiseev, Sergey, Novikov, Pavel, Majewski, Dominik, Stebbings, Simon, Agachi, Svetlana, Limonta, Massimiliano, Francesco Selmi, Carlo, Rezus, Elena, Herrmann, Kristine, Granel, Brigitte, Seskute, Goda, Seidel, Matthia, Hasler, Paul, Cutolo Vera Bernardino, Maurizio, Pizzorni, Carmen, Morovic-Vergles, Jadranka, Furst, Daniel, Ramazan, Ana-Maria, Bajocchi, Gianluigi, Stamp, Lisa, Rimar, Doron, Marcoccia, Antonella, Novak, Srdan, Mouthon, Luc, Stork, Jiri, S Chung, Lorinda, Poormoghim, Hadi, Gaches, Franci, Belloli, Laura, Tanaseanu, Cristina-Mihaela, Atzeni, Fabiola, Eyerich, Kilian, M Hsu, Ivien, van Laar, Jacob, Ellen Csuka, Mary, Nuri Pamuk, Omer, Couto, Maura, Mekinian, Arsene, Inanc, Murat, Foeldvari, Ivan, Martínez-Barrio, Julia, Levy, Yair, Markus, Juliana, and Oliveira, Susana
Subjects: Adult, Male, 0301 basic medicine, medicine.medical_specialty, weight loss, systemic sclerosis, nutrition, Immunology, Disease, computer.software_genre, General Biochemistry, Genetics and Molecular Biology, Scleroderma, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Risk Factors, Weight loss, Internal medicine, Weight Loss, Epidemiology, medicine, Humans, Immunology and Allergy, 610 Medicine & health, Aged, 030203 arthritis & rheumatology, Scleroderma, Systemic, Database, business.industry, Middle Aged, medicine.disease, 030104 developmental biology, Mood, Databases as Topic, Female, Outcomes research, medicine.symptom, business, computer, Rheumatism
Abstract: Gastrointestinal (GI) involvement is almost universal in patients with systemic sclerosis (SSc) and is associated with significant disease-related morbidity and mortality.1 The entire GI tract can be involved and other disease features (eg, low mood, terminal organ failure and functional hand impairment) can result in significant nutritional impairment. Severe GI involvement has been reported to occur in ~10% of patients with SSc and often occurs early in the course of the disease.2 However, identification of patients at high risk of clinically significant weight loss is extremely challenging, including from the high prevalence of GI symptoms in patients with SSc. Therefore, there is a need to understand high-risk patients including potentially modifiable risk factors, with a view to early intervention strategies. Against this background, the aim of this study was to examine potential clinical risk factors of significant weight loss in patients with SSc. We performed an analysis of patients with SSc enrolled in the multinational, longitudinal European League Against Rheumatism (EULAR) Scleroderma Trials and Research (EUSTAR) database. In our study, we defined significant weight loss as 4.5 kg and/or least 5% of their body weight at 5 months onwards.3 Patients with a recorded second visit after 3 months and before 12 months were included in the analysis. We adopted a pragmatic approach (relevant to clinical practice) in …
Published: 2020
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13. Nocardioses au cours des vascularites systémiques

Author: Xavier Puéchal, R. Mestiri, Benjamin Terrier, N. Wesner, Thierry Zenone, C.A. Durel, Groupe français d’étude des vascularites, Karim Sacre, Pierre-Emmanuel Charles, Paul Legendre, François Lifermann, M. Desprets, and Y. Crabol
Subjects: Gastroenterology, Internal Medicine
Abstract: Introduction Les nocardioses sont des infections dues a un bacille gram positif aerobie denomme Actinomycete nocardia, ayant la capacite de disseminer dans n’importe quel organe, en premier lieu le poumon, la peau et le systeme nerveux. Un defaut d’immunite cellulaire et les pathologies broncho-pulmonaires sont les principaux facteurs de risque. Le cotrimoxazole est le traitement privilegie, ce dernier etant souvent utilise en prophylaxie de la pneumocystose chez les patients immunodeprimes. L’objectif de cette etude est de decrire les nocardioses survenant chez les patients atteints de vascularite systemique. Patients et methodes Dans cette etude francaise retrospective multicentrique, nous avons inclus les patients suivis pour une vascularite, selon la nomenclature de Chapel Hill revisee en 2012, et ayant presente une infection a Nocardia spp. au cours de leur suivi. Nous decrivons leurs caracteristiques, les facteurs d’immunosuppression et leur evolution. Resultats Nous avons inclus 14 patients ayant presente une nocardiose au cours du suivi d’une vascularite, de sexe masculin dans 9/14 (64 %) cas et avec un âge median de 82 ans (IQR 73,5–84). Les deux vascularites les plus associees a la survenue d’un nocardiose etaient la granulomatose avec polyangeite (GPA) dans 8 cas (57 %) et l’arterite a cellules geantes (ACG) dans 5 cas (36 %). Toutes les GPA avaient une atteinte pulmonaire (hemorragie intra-alveolaire ou nodules excaves), celle-ci n’etant jamais retrouvee dans les ACG. Les patients avaient tous recus une corticotherapie, initialement a une dose mediane de 60 mg/j (IQR 55–70), associee pour les GPA a du cyclophosphamide dans 6 cas (75 %, dose cumulee mediane de 13,5 g) et/ou du rituximab dans 4 cas (50 %). Seuls 4/14 (29 %) recevaient un traitement prophylactique par cotrimoxazole. Il n’y avait pas d’autres facteurs de risque d’infections en dehors de l’immunodepression induite par le traitement. Les taux medians de gammaglobulines, lymphocytes totaux et CD4+ etaient de 6,45 g/L (5,75–10,3), 920/mm3 (870–1910) et 558/mm3 (389–597). La nocardiose survenait apres une duree mediane de 12 mois (IQR 0-51) d’evolution de la vascularite, sous une dose mediane de prednisone de 20 mg/j (IQR 15–30). Elle etait limitee dans 11/14 (79 %) cas et disseminee dans les 3 autres cas (21 %). La moitie des patients avec GPA avaient une forme pulmonaire de nocardiose, rendant le diagnostic differentiel difficile avec une rechute de la vascularite. Les autres localisations etaient cerebrales dans 7 cas (50 %) et cutanee dans 5 cas (36 %). Le diagnostic reposait sur la culture (12/12, 100 %), la PCR (4/10, 40 %) et/ou l’histologie (2/10, 20 %). Le cotrimoxazole etait la pierre angulaire du traitement chez la majorite des patients (10/14, 71 %), mais une bi- voire tritherapie etait instauree chez 10/14 (71 %) pendant une duree prolongee allant de 3 a 12 mois. Trois patients ont presente une poussee de leur vascularite, dont deux concomitantes a l’infection. La survenue de la Nocardiose a conduit a alleger le traitement immunosuppresseur dans 62 % (8/13) des cas. Une prophylaxie secondaire a ete demarree dans 55 % des cas (6/11). Cinq patients sont decedes au cours du suivi, dont seulement un directement lie a l’infection (atteinte neurologique severe). Conclusion La GPA et l’ACG peuvent se compliquer de nocardiose au cours de leur suivi, en particulier chez les patients âges. Les atteintes pulmonaires de la nocardiose peuvent etre difficiles a differencier de l’atteinte pulmonaire au cours des GPA. Le cotrimoxazole est le traitement de reference mais la dose utilisee en prophylaxie n’est pas adaptee contre la nocardiose.
Published: 2020

14. Risk of autoimmune diseases and human papilloma virus (HPV) vaccines: Six years of case-referent surveillance

Author: Lamiae Grimaldi-Bensouda, Michel Rossignol, Isabelle Koné-Paut, Alain Krivitzky, Christine Lebrun-Frenay, Johanna Clet, David Brassat, Caroline Papeix, Marc Nicolino, Pierre-Yves Benhamou, Olivier Fain, Nathalie Costedoat-Chalumeau, Marie-France Courcoux, Jean-François Viallard, Bertrand Godeau, Thomas Papo, Patrick Vermersch, Isabelle Bourgault-Villada, Gerard Breart, Lucien Abenhaim, Firas Abbas, Abdelhakim Abdelmoumni, Pascal Hilliquin, Elisabeth Requeda, Daniel Adoue, Christian Agard, Agathe Masseau, Nathalie Aladjidi, Helder Fernandes, Gwendal Lemasson, Yves Perel, Isabelle Raymond, Olivier Richer, Anne Vital, Emma Allain-Launay, Marie Bru, Caroline Thomas, Jean-Jacques Altman, Daniel Amsallem, Nazmiye Aras, Latifato Boukari, Marie Dubrel, Edouard Letellier, Nadine Lucidarme, Arsène Mekinian, Anne-Sophie Morin, Jérôme Stirnemann, Catherine Atlan, Dominique Audry, Jérôme Augustin, Redouane Bakir, Pablo Bartolucci, Xavier Chevalier, Constance Guillaud, Mehdi Khellaf, Nicolas Limal, Valentine Lousteau, Matthieu Mahevas, Gayane Méliksetyan, Marc Michel, Mathilde Roumier, Sophie Bayart, Fabrice Bonnet, Olivier Decaux, Amine Bekherraz, Benoit Brihaye, Roger Dachez, Eric Daugas, Gilles Hayem, Olivier Meyer, Elisa Pasqualoni, Karim Sacre, Florence Travert, Hélène Bellon, Jacques Beltrand, François Lefrere, Albane Simon, Olivier Benveniste, Francis Bolgert, Raphael De Paz, Sophie Demeret, Bruno Fautrel, Sophie Jacqueminet, Céline Louapre, Elizabeth Maillart, Nathalie Morel, Julie Rigabert, Philippe Bensaid, Claire Berger, Patrick Berquin, Anne-Gaëlle Le Moing, Stéphane Berroir, Gérard Besson, Célia Boutte, Olivier Casez, Bernard Bonnotte, Sylvain Audia, Cécile Bossu-Estour, Anne Bourgarit, Alain Dupuy, Homa Keshmandt, Bertrand Bourre, Aude Brac, Agnès Perrin, Corinne Pondarré, Sylvie Villar-Fimbel, Isabelle Bruckert, Anne Cosson, Nadine Magy-Bertrand, Guillaume Tisserand, William Camu, Bertrand Carlander, Raul Juntas Morales, Claude Cances, Marlene Pasquet, Maria Angela Castilla Lievre, Stephanie Chabroux, Mamoud Charif, Emmanuel Chatelus, Jean Sibilia, Jacqueline Chevrant-Breton, Sylvaine Clavel, Françoise Bille-Turc, Jacques Cohen, Marie France Courcoux, Guy Leverger, Laurent Machet, Jean-Marie Cuisset, Pascale Cony-Makhoul, Paul Darsy, Sandrine Favre, Pierrick Giraud, Laurence Leitenschenck, Irène Monteiro, Chafika Morati, Jérôme DeSeze, Monica Dinulescu, Taher Dhaoui, Florence Dommange-Romero, Elisabeth Drevard, Clémentine Dupuis, Marie-Laure Dumuis, Jean-Marc Durand, Samia Farad, Pierre Lecomte, Peggy Pierre, Fanny Fouyssac, Philippe Gaudin, Alain Gautier, Justine Gellen-Dautremer, Irène Jarrin, Pascal Richette, Emilie Georget, Pierre Gras, Thibault Moreau, Eric Giraud, Maya Hacini, Anne Mayer, Cécile Guillaumat, Séverine Guillaume, Corinne Guitton, Isabelle Kone-Paut, Céline Marsaud, Linda Rossi, Marie-Hélène Guyot, Patrick Hassler, Claude Heimfert, Olivier Heinzlef, Brigitte Hillion, Catherine Hocquelet, Helene Husson, Pierre Ichai, Eric Jeziorski, Chantal Job Deslandre, Véronique Le Guern, Kamen Kamenov, Véronique Kerlan, Philippe Lemoine, Laurent Misery, Brigitte Pan-Petesch, Pierre Labauge, Michel Rodier, Chadi Lacade, Berthe Razafimahefa, Karim Lachgar, Marie-Pierre Larmarau, Thierry Leblanc, Patrick Lefèbvre, Philippe Lejoyeux, Charles Leske, Kim Ly, Laurent Magy, Sylvie Mansuy, Richard Marechaud, Marie-Laure Martin Negrier, Guilhem Sole, Jean Maupetit, Françoise Mazingue, Stéphanie Mochon, Blidi Moktar, Donald Morcamp, Nathalie Morlet-Barla, Guillaume Nicolas, Vivien Pautot, Isabelle Pellier, Jean-Luc Verret, Olivier Outteryck, Beatrice Pallot-Prades, Jean Michel Paquet, Xavier Puechal, Annie Sortais, Jean Pelletier, Audrey Rico, Dominique Pez, Bruno Stankoff, Philippe Quittet, Claude Rémy, Eléna Roba, Hélène Rosario, Nathalie Roudaut, Emmanuel Sonnet, Michel Ruel, Samuel Sebban, Pauline Schaepelynck, Marie-Jeanne Simonin, Christophe Vial, Jean-Francois Viallard, Isabelle Ladedan, Thierry Zenone, LASER ANALYTICA, Paris (LA-SER), Centre d'enseignement Cnam Paris (CNAM Paris), Conservatoire National des Arts et Métiers [CNAM] (CNAM), HESAM Université - Communauté d'universités et d'établissements Hautes écoles Sorbonne Arts et métiers université (HESAM)-HESAM Université - Communauté d'universités et d'établissements Hautes écoles Sorbonne Arts et métiers université (HESAM), Service de Rhumatologie [CHU Bicêtre], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre, Centre de référence des maladies auto-inflammatoires, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de médecine interne [Avicenne], Hôpital Avicenne [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Neurologie [CHU Nice], Hôpital Pasteur [Nice] (CHU)-Centre Hospitalier Universitaire de Nice (CHU Nice), CHU Bordeaux [Bordeaux], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Hôpital Femme Mère Enfant [CHU - HCL] (HFME), Hospices Civils de Lyon (HCL), CHU Grenoble, Service de médecine interne [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Service de médecine interne et centre de référence des maladies rares [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], CHU Trousseau [APHP], Service de médecine interne et maladies infectieuses [Bordeaux], CHU Bordeaux [Bordeaux]-Groupe hospitalier Saint-André, Service de médecine interne [Mondor], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), AP-HP - Hôpital Bichat - Claude Bernard [Paris], Centre de recherche sur l'Inflammation (CRI (UMR_S_1149 / ERL_8252 / U1149)), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Département Hospitalo-Universitaire Fibrosis, Inflammation, Remodeling in cardiovascular, respiratory and renal diseases (Paris), Lille Inflammation Research International Center - U 995 (LIRIC), Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Hôpital Claude Huriez [Lille], CHU Lille, Université de Versailles Saint-Quentin-en-Yvelines (UVSQ), Institut Cochin (IC UM3 (UMR 8104 / U1016)), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Hôpital Ambroise Paré [AP-HP], Equipe 1 : EPOPé - Épidémiologie Obstétricale, Périnatale et Pédiatrique (CRESS - U1153), Université Paris Descartes - Paris 5 (UPD5)-Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité (CRESS (U1153 / UMR_A_1125 / UMR_S_1153)), Institut National de la Recherche Agronomique (INRA)-Université Paris Diderot - Paris 7 (UPD7)-Université Paris Descartes - Paris 5 (UPD5)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Recherche Agronomique (INRA)-Université Paris Diderot - Paris 7 (UPD7)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM), DHU Risques Et Grossesse, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), CHU Toulouse [Toulouse], Service de neurologie 1 [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service d'hématologie-immunologie-oncologie pédiatrique [CHU Trousseau], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre National de la Recherche Scientifique (CNRS)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité (CRESS (U1153 / UMR_A_1125 / UMR_S_1153)), Institut National de la Recherche Agronomique (INRA)-Université Paris Diderot - Paris 7 (UPD7)-Université Paris Descartes - Paris 5 (UPD5)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Recherche Agronomique (INRA)-Université Paris Diderot - Paris 7 (UPD7)-Université Paris Descartes - Paris 5 (UPD5)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Bicêtre, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Hôpital Avicenne, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Pitié-Salpêtrière [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP], Service d’oncologie hématologie pédiatrique [CHU Trousseau], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Diderot - Paris 7 (UPD7), Lille Inflammation Research International Center (LIRIC), Hôpital Claude Huriez, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Hôpital Ambroise Paré, Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Diderot - Paris 7 (UPD7)-Centre National de la Recherche Scientifique (CNRS), Service de Neurologie [CHU Pitié-Salpêtrière], IFR70-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP), and Centre National de la Recherche Scientifique (CNRS)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)
Subjects: Adult, Male, Risk, Pediatrics, medicine.medical_specialty, Adolescent, Immunology, HPV vaccines, Autoimmune Diseases, Autoimmune thyroiditis, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Autoimmune disease, Odds Ratio, medicine, Humans, Immunology and Allergy, Papillomavirus Vaccines, 030212 general & internal medicine, Family history, Young adult, Child, HPV vaccine, business.industry, Pharmacoepidemiology, Papillomavirus Infections, Odds ratio, medicine.disease, Thrombocytopenic purpura, Connective tissue disease, Confidence interval, 3. Good health, Population Surveillance, [SDV.IMM]Life Sciences [q-bio]/Immunology, Female, business, 030217 neurology & neurosurgery, Follow-Up Studies
Abstract: Background Safety of HPV vaccines is still in question due to reports of autoimmune diseases (ADs) following HPV immunization. Objectives To assess the risk of ADs associated with HPV vaccination of female adolescents/young adults in France. Methods Systematic prospective case-referent study conducted to assess the risks associated with real-life use of HPV vaccines. Cases were female 11–25 years old with incident ADs [central demyelination/multiple sclerosis (CD/MS), connective tissue disease (CTD), Guillain-Barre syndrome (GBS), type-1 diabetes (T1D), autoimmune thyroiditis (AT), and idiopathic thrombocytopenic purpura (ITP)]. Cases were consecutively and prospectively identified at specialized centers across France (2008–2014) and individually matched by age and place of residence to referents recruited in general practice. Risk was computed using multivariate conditional logistic regression models adjusted for family history of ADs, living in France (north/south), co-medications and co-vaccinations. Results With a total of 478 definite cases matched to 1869 referents, all ADs combined were negatively associated to HPV vaccination with an adjusted odds ratio of 0.58 (95% confidence interval: 0.41–0.83). Similar results were obtained for CD/MS, AT, CT, and T1D, the last two not reaching statistical significance. No association was found for ITP and GBS. Sensitivity analyses combining definite and possible cases with secondary time window showed similar results. Conclusion Exposure to HPV vaccines was not associated with an increased risk of ADs within the time period studied. Results were robust to case definitions and time windows of exposure. Continued active surveillance is needed to confirm this finding for individual ADs.
Published: 2017

15. Rituximab associated vasculitis flare: incidence, predictors and outcome

Author: Jean-Charles Piette, Bertrand Lioger, Philippe Rouvier, Anne Claire Desbois, Matthieu Resche-Rigon, Patrice Cacoub, Lucie Biard, Thierry Zenone, Neila Benameur, Sophie Candon, Isabelle Brocheriou, David Saadoun, Lucile Musset, Damien Sène, Immunologie - Immunopathologie - Immunothérapie [CHU Pitié Salpêtrière] (I3), CHU Charles Foix [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service d'anatomie pathologique [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Médecine Interne (VALENCE - Med Int), Centre hospitalier de Valence, Service de Département de médecine interne et immunologie clinique [CHU Pitié-Salpêtrière] (DMIIC), CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Laboratoire de Synthèse et Fonctionnalisation de Céramiques (LSFC), Saint Gobain-Institut de Chimie du CNRS (INC)-Centre National de la Recherche Scientifique (CNRS), Université Pierre et Marie Curie - Paris 6 - UFR de Médecine Pierre et Marie Curie (UPMC), Université Pierre et Marie Curie - Paris 6 (UPMC), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de médecine interne et d'immunologie clinique [CHU Pitié-Salpêtrière], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)
Subjects: Vasculitis, medicine.medical_specialty, Myocarditis, [SDV]Life Sciences [q-bio], Immunology, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Internal medicine, hemic and lymphatic diseases, medicine, Humans, Immunology and Allergy, 030212 general & internal medicine, Survival rate, Retrospective Studies, 030203 arthritis & rheumatology, Autoimmune disease, business.industry, Incidence, Mortality rate, medicine.disease, Cryoglobulinemia, 3. Good health, Purpura, Treatment Outcome, [SDV.IMM]Life Sciences [q-bio]/Immunology, Rituximab, medicine.symptom, business, medicine.drug
Abstract: Objective.To report the incidence, predictors, and outcome of rituximab (RTX)-associated autoimmune disease flare.Methods.We conducted a retrospective study in a tertiary referral center from 2005 to 2015. Disease flare was defined as the onset of a new organ involvement or worsening of autoimmune disease within 4 weeks following RTX.Results.Among the 185 patients, we identified 7 disease flares (3.4%). All were due to type II mixed cryoglobulinemia vasculitis. Vasculitis flare occurred after a median time of 8 days (range 2–16) following RTX infusion and included acute kidney insufficiency (n = 7), purpura with cutaneous (n = 7), gastrointestinal (GI) tract involvement (n = 4), and myocarditis (n = 1). Patients with RTX-associated cryoglobulinemia vasculitis flare had these conditions more frequently: renal involvement (p = 0.0008), B cell lymphoproliferation (p = 0.015), higher level of cryoglobulin (2.1 vs 0.4 g/l, p = 0.0004), and lower level of C4 (0.02 vs 0.05, p = 0.023) compared to patients without flare after RTX (n = 43). Four patients (57%) died after a median time of 3.3 months. The 1-year survival rate was poorer in patients with vasculitis flare after RTX compared to their negative counterpart [43% (95% CI 18–100) vs 97% (95% CI 92–100), p < 0.001]. Immunofluorescence analysis of kidney biopsy in patients with worsening RTX-associated vasculitis highlighted the presence of RTX-, IgM-, and IgG1-positive staining of endomembranous deposits and thrombi within kidney lesions.Conclusion.RTX-associated cryoglobulinemia vasculitis flare is associated with high mortality rate. We provided evidence that kidney lesions are due to immune complex deposition and to glomerular obstruction by cryoglobulinemia and RTX.
Published: 2019

16. Large-vessel vasculitis diagnosed between 50 and 60 years: Case-control study based on 183 cases and 183 controls aged over 60 years

Author: Hervé Devilliers, Loïc Guillevin, Daniel Engelbert Blockmans, Alexis Régent, Kevin Bouiller, Olivier Espitia, Alice Bérezné, Christian Agard, Maxime Samson, Laure Delaval, Yann Nguyen, Alexandre Balageas, Mélanie Roriz, Karim Sacre, Alban Deroux, Hubert de Boysson, Aurélie Daumas, Sébastien Humbert, Stéphane Vinzio, M. Puyade, V. Grobost, Estibaliz Lazaro, François Maurier, Grégory Pugnet, O. Souchaud-Debouverie, Virginie Rieu, Henry Dupuy, Claire de Moreuil, Thierry Zenone, Cécile-Audrey Durel, Ygal Benhamou, Laurent Arnaud, Eric Liozon, Mikael Ebbo, François Lifermann, Benjamin Terrier, Aix Marseille Université (AMU), Interactions hôte-greffon-tumeur, ingénierie cellulaire et génique - UFC (UMR INSERM 1098) (RIGHT), Institut National de la Santé et de la Recherche Médicale (INSERM)-Etablissement français du sang [Bourgogne-Franche-Comté] (EFS BFC)-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC), Hôpital de la Timone [CHU - APHM] (TIMONE), Service de médecine interne [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Service de Médecine interne A et polyclinique médicale [CHU Limoges], CHU Limoges, Hôpital Haut-Lévêque [CHU Bordeaux], CHU Bordeaux [Bordeaux], Centre hospitalier universitaire de Poitiers (CHU Poitiers), University Hospital Gasthuisberg [Leuven], Service de Médecine Interne [CHU Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Service de Médecine Interne, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital Cochin [AP-HP], Service de médecine interne et maladies systémiques (SOC 2) [CHU de Dijon], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Service de médecine interne, Hôpital Sainte-Blandine-Centre hospitalier régional Metz-Thionville (CHR Metz-Thionville), Centre hospitalier de Valence, Département de Médecine Interne et Pneumologie [Brest] (DMIP - Brest), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Service de Médecine Interne [Dax], Centre Hospitalier de Dax, Sarcomes osseux et remodelage des tissus calcifiés - Phy-Os [Nantes - INSERM U1238] (Phy-Os), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Bretagne Loire (UBL)-Centre hospitalier universitaire de Nantes (CHU Nantes)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN), Centre Hospitalier Universitaire [Grenoble] (CHU), CHU Clermont-Ferrand, Service de médecine interne [Nantes], Université de Nantes (UN)-Hôtel-Dieu-Centre hospitalier universitaire de Nantes (CHU Nantes), Centre hospitalier de Pau, Laboratoire Chrono-environnement (UMR 6249) (LCE), Centre National de la Recherche Scientifique (CNRS)-Université de Franche-Comté (UFC), service de maladies infectieuses CHU J Minjoz Besancon, Service de Médecine Interne [Hôpital Edouard Herriot - HCL], Hôpital Edouard Herriot [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Chirurgie otologique mini-invasive robotisée, Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut Cochin (IC UM3 (UMR 8104 / U1016)), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université Paris Descartes - Paris 5 (UPD5), Immunologie - Immunopathologie - Immunothérapeutique (I3), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC)-Etablissement français du sang [Bourgogne-Franche-Comté] (EFS [Bourgogne-Franche-Comté]), University Hospital Gasthuisberg, CHU Toulouse [Toulouse], Centre hospitalier régional Metz-Thionville (CHR Metz-Thionville)-Hôpital Sainte-Blandine, Laboratoire Chrono-environnement - CNRS - UBFC (UMR 6249) (LCE), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Institut National de la Santé et de la Recherche Médicale (INSERM)-Etablissement français du sang [Bourgogne-Franche-Comté] (EFS [Bourgogne-Franche-Comté])-Université de Franche-Comté (UFC), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN)-Centre hospitalier universitaire de Nantes (CHU Nantes)-Université Bretagne Loire (UBL), Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), and Centre National de la Recherche Scientifique (CNRS)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)
Subjects: 0301 basic medicine, Male, medicine.medical_specialty, Immunology, Takayasu arteritis, Giant Cell Arteritis, Outcome measures, 03 medical and health sciences, 0302 clinical medicine, Large vessel vasculitis, Refractory disease, Immunology and Allergy, Medicine, Humans, cardiovascular diseases, Age of Onset, 10. No inequality, skin and connective tissue diseases, Large-vessel vasculitis, Aged, Retrospective Studies, 030203 arthritis & rheumatology, Aged, 80 and over, business.industry, Refractory Disease, Case-control study, Middle Aged, medicine.disease, Dermatology, 3. Good health, Giant cell arteritis, 030104 developmental biology, Case-Control Studies, cardiovascular system, Female, business, Vasculitis, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract: Age at onset of large-vessel vasculitis (LVV) is commonly used to distinguish giant cell arteritis (GCA) and Takayasu arteritis (TA). However, LVV between age 50 and 60 years may be difficult to classify.We conducted a retrospective study including LVV aged between 50 and 60 years at onset (LVVWe included 183 LVV
Published: 2019

17. Chronic obstructive pulmonary disease associated with hypocomplementemic urticarial vasculitis

Author: Daniel Engelbert Blockmans, C. David, Anne Peterschmitt, Thierry Zenone, Delphine Gobert, Marie Jachiet, Benjamin Terrier, S. Palat, Achille Aouba, Anne Sophie Gamez, Luc Mouthon, Marc Pineton de Chambrun, François Lifermann, Anas Mehdaoui, Séverine Feuillet-Soummer, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Centre Hospitalier Eure-Seine - Hôpital d'Evreux - Vernon (Evreux), Service de médecine interne [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), and Université de Paris (UP)
Subjects: medicine.medical_specialty, Urticaria, business.industry, Hypocomplementemic urticarial vasculitis, [SDV]Life Sciences [q-bio], MEDLINE, Pulmonary disease, medicine.disease, Dermatology, Pulmonary Disease, Chronic Obstructive, 03 medical and health sciences, 0302 clinical medicine, 030228 respiratory system, medicine, Humans, Vasculitis, Leukocytoclastic, Cutaneous, Immunology and Allergy, 030212 general & internal medicine, business, Vasculitis
Published: 2020

18. Sévérité de l’atteinte bronchique aux cours des vascularites urticariennes hypocomplémentémique

Author: J. Marie, A. Le Quellec, Benjamin Terrier, A. Peterschmitt, S. Pallat, Albertine Aouba, François Lifermann, Anas Mehdaoui, G. Delphine, Thierry Zenone, M. Pineton De Chambrun, Daniel Engelbert Blockmans, and C. David
Subjects: Gastroenterology, Internal Medicine
Abstract: Introduction La vascularite urticarienne hypocomplementemique (VUH) est un syndrome rare associant une urticaire recidivante, une vascularite leucocytoclasique sur la biopsie cutanee et une hypocomplementemie. L’atteinte d’autres organes est frequente. Parmi ces manifestations extracutanees, une atteinte pulmonaire specifique a type de trouble ventilatoire obstructif (TVO) a ete decrite mais les donnees sur cette atteinte sont exceptionnelles. Nous rapportons les caracteristiques de l’atteinte pulmonaire chez les patients atteints de VUH. Patients et methodes Cette etude retrospective multicentrique a inclus des patients ayant un diagnostic de VUH dont le diagnostic a ete pose entre 2004 et 2019, et un trouble ventilatoire obstructif defini sur les epreuves fonctionnelles respiratoires par un rapport VEMS/CVF Resultats Nous avons inclus 10 patients (8 femmes et 2 hommes), avec un âge median de 60,5 (39–77) ans. La VUH etait isolee dans 9 cas (90 %) et associee a une thyroidite d’Hashimoto dans 1 cas (10 %). L’atteinte cutanee consistait en une urticaire associee a des angio-œdemes dans 80 % et un purpura dans 30 %. Les autres manifestations extracutanees etaient : atteinte ophtalmologique dans 70 %, signes generaux dans 60 %, atteinte renale dans 20 % et atteinte digestive dans 20 %. Sur le plan pulmonaire, les patients etaient symptomatiques dans tous les cas, avec une dyspnee retrouvee dans 90 %, une toux dans 50 % et une spasticite dans 70 %. Quatre-vingt-dix pour cent des patients etaient fumeurs actifs ou sevres, avec une consommation mediane estimee a 20 (5–40) paquets-annees. Le VEMS/CV median au diagnostic du TVO etait de 60 (27–70) % avec un VEMS median a 45 (27–81) %. Le TDM thoracique etait anormal dans 70 % des cas, retrouvant comme principales anomalies : emphyseme dans 57 %, dilatation des bronches dans 57 %, perfusion mosaique dans 28 %. Le nombre median de lignes therapeutiques recues par ces patients etait de 4 (2–6) temoignant du caractere severe et refractaire. L’ensemble des patients ont recu une corticotherapie avec une dose mediane de 45 (20–80) mg/j. Parmi les immunosuppresseurs recus, on retrouvait : hydroxychloroquine dans 80 %, colchicine ou dapsone dans 40 %, mycophenolate mofetil dans 60 %, cyclophosphamide dans 40 %, rituximab dans 40 %, azathioprine dans 30 % et ciclosporine dans 10 %. Seuls 2 patients n’ont pas recu de traitement immunosuppresseurs. Apres un suivi median de 111 (33–211) mois, 60 % avait ete hospitalise en urgence pour une exacerbation, dont un tiers en reanimation. La moitie des patients ont presente une pneumopathie infectieuse, 20 % ont necessite une oxygenotherapie de longue duree et 30 % la mise en place d’une ventilation non-invasive. Au dernier suivi, le VEMS median etait de 32 (20–81) %, 2 patients etaient inscrits sur liste de transplantation pulmonaire, et une patiente etait recusee de l’inscription sur liste du fait de l’etat general. Enfin, deux patientes sont decedees, une de l’evolution de son TVO et l’autre d’une cardiopathie ischemique. Le VEMS median au dernier suivi pour les patients vivants etait de 41 (20–81) %. Conclusion Les troubles ventilatoires obstructifs au cours des VUH sont dans la grande majorite tres severes et surviennent chez des patients fumeurs. Cette atteinte est refractaire a la plupart des traitements immunosuppresseurs, avec un risque important de deces ou la necessite de recourir a la transplantation pulmonaire. L’arret du tabac au cours du diagnostic de VUH semble ainsi etre une priorite absolue vue la severite de cette atteinte.
Published: 2019

19. Smoking in Systemic Sclerosis: a Longitudinal European Scleroderma Trials and Research Group Study

Author: Jaeger, Vk, Valentini, G, Hachulla, E, Cozzi, F, Distler, O, Airó, P, Czirják, L, Allanore, Y, Siegert, E, Rosato, E, Matucci-Cerinic, M, Caimmi, C, Henes, J, Carreira, Pe, Smith, V, Del Galdo, F, Denton, Cp, Ullman, S, Langhe, E, Riccieri, V, Alegre-Sancho, Jj, Rednic, S, Müller-Ladner, U, Walker, Ua, Ananieva, Lidia P., Florenzo, Iannone, Distler, JeorgH. W., Eduardo, Kerzberg, Bojana, Stamenkovic, José Antonio Pereira Da Silva, Ivan, Catellvì, Suleyavuz, Kamal, Solanki, Branimir, Anic, Esthela, Loyo, Tim, Schmeiser, Ruxandra Maria Ionescu, Peter, Villiger, Francesco Paolo Cantatore, Paloma Garcia de la Pena Lefebvre, Carina, Mihai, Dominique Farge Bancel, Pierluigi, Meroni, Thierry, Martin, Stefan, Heitmann, Nicolas, Hunzelmann, Fahrettin, Oksel, Washington, Bianchi, Sara, Bongiovanni, Radim, Becvar, Thierry, Zenone, Alexandra, Balbir-Gurman, Piotr, Wiland, Pawel, Zebryk, Francßoisspertini, Lori, Chung, Alessandra, Vacca, Carlo, Chizzolini, Carlo Francesco Selmi, Cristina-Mihaela, Tanaseanu, Ira, Litinsky, Houssiau, Frederic A., Brigitte, Krummel-Lorenz, Hsu, Vivien M., Francisca, Fontes, Alexandra Maria Burlui, Duska, Martinovic, Sergei, Popa, Itzhak, Rosner, Carmel, Mallia, Rosario, Foti, Luc, Mouthon, Martin, Aringer, Jacob van Laar, Roger, Hesselstrand, Maria Rosa Pozzi, Gabriela, Riemekasten, Cutolo, Maurizio, Paul, Hasler, Zea Mendoza Antonio, Cord, Sunderkotter, Codrina, Ancuta, Jean, Sibilia, Roberto, Caporali, Armando, Gabrielli, Raffaele, Pellerito, Ariane, Herrick, Øyvind, Midtvedt, Kilian, Eyerich, Brigitte, Granel, Lesley Ann Saketkoo, Maura, Couto, Yael, Eizikovits, and Giuseppina, Abignano.
Published: 2018

20. Cogan syndrome: Characteristics, outcome and treatment in a French nationwide retrospective study and literature review

Author: Robin Dhote, Christophe Deligny, Charlotte Durtette, Edouard Pertuiset, Christian Lavigne, Anne Grasland, Thomas Quemeneur, Eric Hachulla, Thomas Papo, Pascal Sève, Matthieu Resche-Rigon, Pierre-Yves Hatron, Thierry Zenone, Marc Lambert, Arsène Mekinian, Thomas Le Gallou, Benoit De Wazieres, Mohamed Hamidou, Guillaume Gondran, Cédric Landron, Bertrand Lioger, Jacques Pouchot, Jean Emmanuel Kahn, Snfmi, Olivier Fain, Service de Médecine Interne [CHU Saint-Antoine], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Saint-Antoine [APHP], Service de rhumatologie, inflammation-immunopathologie- biothérapie [CHU Saint-Antoine] (DHU i2B ), CHU Saint-Antoine [APHP]-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Sorbonne Université - Faculté de Médecine (SU FM), Sorbonne Université (SU), Service de médecine interne [Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de biostatistiques et information médicale [Saint-Louis], Université Paris Diderot - Paris 7 (UPD7)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), AP-HP - Hôpital Bichat - Claude Bernard [Paris], Centre hospitalier de Valence, Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), Service de Médecine interne [CHU de Fort-de France], CHU de Fort de France, Centre hospitalier universitaire de Poitiers (CHU Poitiers), Service de Médecine Interne [CHU HEGP], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Centre de référence des syndromes drépanocytaires majeurs, Université Paris Descartes - Paris 5 (UPD5), Université Sorbonne Paris Cité (USPC), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Hôpital Foch [Suresnes], Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Centre Hospitalier Régional Universitaire de Nîmes (CHRU Nîmes), Université de Montpellier (UM), Service de médecine interne [Avicenne], Hôpital Avicenne, Service de Médecine interne A et polyclinique médicale [CHU Limoges], CHU Limoges, Centre Hospitalier René Dubos [Pontoise], CH Valenciennes, Service de médecine interne [Nantes], Université de Nantes (UN)-Hôtel-Dieu-Centre hospitalier universitaire de Nantes (CHU Nantes), Hôpital de la Croix-Rousse [CHU - HCL], Hospices Civils de Lyon (HCL), Hopital Louis Mourier - AP-HP [Colombes], CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de rhumatologie [CHU Saint-Antoine], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de référence des syndromes drépanocytaires majeurs-Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Hôpital Avicenne [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital Louis Mourier - AP-HP [Colombes], and Centre Hospitalier Régional Universitaire de Tours (CHRU Tours)
Subjects: Adult, Male, medicine.medical_specialty, Cogan's syndrome, Adolescent, Interstitial keratitis, Cogan syndrome, Immunology, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Internal medicine, medicine, Immunology and Allergy, Cogan Syndrome, Humans, 030223 otorhinolaryngology, Prospective cohort study, Child, Aged, Retrospective Studies, Outcome, 030203 arthritis & rheumatology, Keratitis, business.industry, Tumor Necrosis Factor-alpha, Bilateral hearing loss, Retrospective cohort study, Odds ratio, Middle Aged, medicine.disease, Confidence interval, Infliximab, 3. Good health, Surgery, Treatment, Treatment Outcome, Antirheumatic Agents, Child, Preschool, [SDV.IMM]Life Sciences [q-bio]/Immunology, Female, business, medicine.drug
Abstract: Background Cogan syndrome is mainly treated with steroids. We aimed to determine the place of DMARDs and biologic-targeted treatments. Patients and methods We conducted a French nationwide retrospective study of patients with Cogan syndrome (n = 40) and a literature review of cases (n = 22) and analyzed the efficacy of disease-modifying anti-rheumatic drugs (DMARDs) and tumor necrosis factor α (TNF-α) antagonists. Results We included 62 patients (31 females) (median age 37 years [range 2–76]. At diagnosis, 61 patients (98%) had vestibulo-auditory symptoms, particularly bilateral hearing loss in 41% and deafness in 31%. Ocular signs were present in 57 patients (92%), with interstitial keratitis in 31 (51%). The first-line treatment consisted of steroids alone (n = 43; 70%) or associated with other immunosuppressive drugs (n = 18; 30%). Overall, 13/43 (30%) and 4/18 (22%) patients with steroids alone and with associated immunosuppressive drugs, respectively (p = 0.8), showed vestibulo-auditory response; 32/39 (82%) and 15/19 (79%) ocular response; and 23/28 (82%) and 10/14 (71%) general response. Overall 61 patients had used a total of 126 lines of treatment, consisting of steroids alone (n = 51 lines), steroids with DMARDs (n = 65) and infliximab (n = 10). Vestibulo-auditory response was significantly more frequent with infliximab than DMARDs or steroids alone (80% vs 39% and 35%, respectively), whereas ocular, systemic and acute-phase reactant response rates were similar. Infliximab was the only significant predictor of vestibulo-auditory improvement (odds ratio 20.7 [95% confidence interval 1.65; 260], p = 0.019). Conclusion Infliximab could lead to vestibulo-auditory response in DMARDS and steroid-refractory Cogan syndrome, but prospective studies are necessary.
Published: 2017

21. Central nervous system involvement in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): Report of 26 patients and review of the literature

Author: David Launay, Valentine Loustau, Nathalie Costedoat-Chalumeau, Robin Dhote, François Maurier, Vincent Cottin, Edouard Pertuiset, Xavier Puéchal, Luc Mouthon, Aurélie Foucher, Jorg Dieter Seebach, Raphaël André, H. Gil, Boris Bienvenu, Jean-Luc Saraux, Thierry Zenone, Loïc Guillevin, Gilles Blaison, Pascal Cathébras, Joëlle Lapoirie, and Benjamin Terrier
Subjects: Adult, Male, Pathology, medicine.medical_specialty, Subarachnoid hemorrhage, Retinal Artery Occlusion, Immunology, 03 medical and health sciences, 0302 clinical medicine, Eosinophilia, medicine, Immunology and Allergy, Humans, Optic neuritis, Stroke, Aged, Retrospective Studies, 030203 arthritis & rheumatology, Intracerebral hemorrhage, ddc:616, business.industry, Cortical blindness, Cranial nerves, Granulomatosis with Polyangiitis, Brain, Middle Aged, medicine.disease, Prognosis, Dermatology, Asthma, Peripheral neuropathy, Treatment Outcome, Female, business, Granulomatosis with polyangiitis, 030217 neurology & neurosurgery
Abstract: Background Although peripheral nervous system involvement is common in eosinophilic granulomatosis with polyangiitis (EGPA), central nervous system (CNS) manifestations are poorly described. This study aimed to describe CNS involvement in EGPA. Patients and methods This retrospective, observational, multicenter study included patients with EGPA and CNS involvement affecting cranial nerves, brain and/or spinal cord. We also undertook a systematic literature review. Results We analyzed 26 personal cases and 62 previously reported cases. At EGPA diagnosis, asthma was noted in 97%, eosinophilia in 98%, peripheral neuropathy in 55% and cardiac involvement in 41%. 38/71 (54%) were ANCA-positive, with a perinuclear-labeling pattern and/or anti-MPO specificity. CNS was involved in 86% at EGPA diagnosis, preceded EGPA in 2%, and occurred during follow-up in 12% after a median of 24 months. Main neurological manifestations were ischemic cerebrovascular lesions in 46 (52%), intracerebral hemorrhage and/or subarachnoid hemorrhage in 21 (24%), loss of visual acuity in 28 (33%) (15 with optic neuritis, 9 with central retinal artery occlusion, 4 with cortical blindness), and cranial nerves palsies in 18 (21%), with 25 patients having ≥ 1 of these clinical CNS manifestations. Among the 81 patients with assessable neurological responses, 43% had complete responses without sequelae, 43% had partial responses with long-term sequelae and 14% refractory disease. After a mean follow-up of 36 months, 11 patients died including 5 from intracerebral hemorrhages. Conclusion EGPA-related CNS manifestations form 4 distinct neurological pictures: ischemic lesions, intracerebral hemorrhages, cranial nerve palsies and loss of visual acuity. Such manifestation should prompt practitioners to consider EGPA in such conditions. Long-term neurological sequelae were common, and intracerebral hemorrhages had the worst prognostic impact.
Published: 2017

22. Mapping and predicting mortality from systemic sclerosis

Author: Kamal Solanki, Roger Hesselstrand, Ulrich A Walker, Jiri Stork, Carlo Chizzolini, Stefan Heitmann, Vanessa Smith, Maurizio Cutolo, Simona Rednic, Carolina de Souza Müller, Jörg H W Distler, Luc Mouthon, Nicolas Hunzelmann, Edoardo Rosato, Mohammed Tikly, Franco Cozzi, Nemanja Damjanov, Paola Caramaschi, Florenzo Iannone, Sule Yavuz, Alexandra Balbir-Gurman, Marco Matucci-Cerinic, Duska Martinovic, Carlos Alberto Von Mühlen, Beatriz Joven, Carina Mihai, Oliver Distler, Paolo Airò, Emmanuel Chatelus, Paloma García de la Peña Lefebvre, Gabriela Riemekasten, Grégoire Rey, Marouane Boubaya, Jörg Henes, Alessandra Vacca, Ulf Müller-Ladner, Thierry Zenone, Armando Gabrielli, Srdan Novak, Ellen De Langhe, Juan José Alegre-Sancho, Walter Alberto Sifuentes-Giraldo, R. Becvar, Jacob M van Laar, Matthias Seidel, Carlomaurizio Montecucco, Daniela Opris, Esthela Loyo, Kilian Eyerich, Vera Ortiz Santamaria, Otylia Kowal-Bielecka, L. Ananieva, Muriel Elhai, Valeria Riccieri, Vanesa Cosentino, Christophe Meune, Panayiotis G. Vlachoyiannopoulos, Jérôme Avouac, Maria De Santis, Branimir Anić, Eric Hachulla, Susanne Ullman, Francesca Ingegnoli, Gabriella Szücs, Bojana Stamenkovic, Yannick Allanore, Serena Vettori, Maria João Salvador, László Czirják, Lisa K. Stamp, Elhai, Muriel, Meune, Christophe, Boubaya, Marouane, Avouac, Jã©rã´me, Hachulla, Eric, Balbir gurman, Alexandra, Riemekasten, Gabriela, Airã², Paolo, Joven, Beatriz, Vettori, Serena, Cozzi, Franco, Ullman, Susanne, Czirjã¡k, Lã¡szlã³, Tikly, Mohammed, Mã¼ller ladner, Ulf, Caramaschi, Paola, Distler, Oliver, Iannone, Florenzo, Ananieva, Lidia P, Hesselstrand, Roger, Becvar, Radim, Gabrielli, Armando, Damjanov, Nemanja, Salvador, Maria J, Riccieri, Valeria, Mihai, Carina, Szã¼cs, Gabriella, Walker, Ulrich A, Hunzelmann, Nicola, Martinovic, Duska, Smith, Vanessa, Mã¼ller, Carolina De Souza, Montecucco, Carlo Maurizio, Opris, Daniela, Ingegnoli, Francesca, Vlachoyiannopoulos, Panayiotis G, Stamenkovic, Bojana, Rosato, Edoardo, Heitmann, Stefan, Distler, Jã¶rg H. W, Zenone, Thierry, Seidel, Matthia, Vacca, Alessandra, Langhe, Ellen De, Novak, Srdan, Cutolo, Maurizio, Mouthon, Luc, Henes, Jã¶rg, Chizzolini, Carlo, Mã¼hlen, Carlos Alberto Von, Solanki, Kamal, Rednic, Simona, Stamp, Lisa, Anic, Branimir, Santamaria, Vera Ortiz, Santis, Maria De, Yavuz, Sule, Sifuentes giraldo, Walter Alberto, Chatelus, Emmanuel, Stork, Jiri, Laar, Jacob Van, Loyo, Esthela, De La Peã±a Lefebvre, Paloma Garcia, Eyerich, Kilian, Cosentino, Vanesa, Alegre sancho, Juan Jose, Kowal bielecka, Otylia, Rey, Grã©goire, Matucci cerinic, Marco, and Allanore, Yannick
Subjects: pulmonary fibrosi, 0301 basic medicine, Male, Genetics and Molecular Biology (all), Time Factors, Databases, Factual, systemic sclerosis, Disease, Biochemistry, Scleroderma, 0302 clinical medicine, cardiovascular disease, Risk Factors, Cause of Death, Epidemiology, Immunology and Allergy, skin and connective tissue diseases, Cause of death, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti. Interna medicina, ddc:616, education.field_of_study, Framingham Risk Score, integumentary system, Orvostudományok, Middle Aged, Prognosis, 3. Good health, Quartile, Cohort, epidemiology, Female, France, systemic sclerosi, pulmonary fibrosis, Aged, Death Certificates, Humans, Proportional Hazards Models, Scleroderma, Systemic, Rheumatology, Immunology, Biochemistry, Genetics and Molecular Biology (all), Human, medicine.medical_specialty, Time Factor, Prognosi, Population, Klinikai orvostudományok, General Biochemistry, Genetics and Molecular Biology, 03 medical and health sciences, Databases, Internal medicine, cardiovascular disease, epidemiology, pulmonary fibrosis, systemic sclerosis, medicine, education, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences. Internal Medicine, Factual, 030203 arthritis & rheumatology, Proportional hazards model, business.industry, Risk Factor, Systemic, Surgery, 030104 developmental biology, Death Certificate, Proportional Hazards Model, business
Abstract: ObjectivesTo determine the causes of death and risk factors in systemic sclerosis (SSc).MethodsBetween 2000 and 2011, we examined the death certificates of all French patients with SSc to determine causes of death. Then we examined causes of death and developed a score associated with all-cause mortality from the international European Scleroderma Trials and Research (EUSTAR) database. Candidate prognostic factors were tested by Cox proportional hazards regression model by single variable analysis, followed by a multiple variable model stratified by centres. The bootstrapping technique was used for internal validation.ResultsWe identified 2719 French certificates of deaths related to SSc, mainly from cardiac (31%) and respiratory (18%) causes, and an increase in SSc-specific mortality over time. Over a median follow-up of 2.3 years, 1072 (9.6%) of 11 193 patients from the EUSTAR sample died, from cardiac disease in 27% and respiratory causes in 17%. By multiple variable analysis, a risk score was developed, which accurately predicted the 3-year mortality, with an area under the curve of 0.82. The 3-year survival of patients in the upper quartile was 53%, in contrast with 98% in the first quartile.ConclusionCombining two complementary and detailed databases enabled the collection of an unprecedented 3700 deaths, revealing the major contribution of the cardiopulmonary system to SSc mortality. We also developed a robust score to risk-stratify these patients and estimate their 3-year survival. With the emergence of new therapies, these important observations should help caregivers plan and refine the monitoring and management to prolong these patients’ survival.
Published: 2017

23. Characteristics of cerebrovascular accidents at time of diagnosis in a series of 98 patients with giant cell arteritis

Author: Marie Puget and Thierry Zenone
Subjects: Male, medicine.medical_specialty, Pediatrics, Poor prognosis, Giant Cell Arteritis, Immunology, Disease, Cohort Studies, Rheumatology, Internal medicine, Humans, Immunology and Allergy, Medicine, Stroke, Vertebral Artery, Aged, Retrospective Studies, Aged, 80 and over, business.industry, Incidence, Brain, Middle Aged, Prognosis, medicine.disease, Magnetic Resonance Imaging, Inflammatory biomarkers, Surgery, Giant cell arteritis, Female, Tomography, X-Ray Computed, Complication, business, Vasculitis, Follow-Up Studies
Abstract: The objective of this study was to determine the characteristics of cerebrovascular accidents at time of diagnosis in patients with giant cell arteritis. Retrospective data were collected from 98 patients at a single hospital with giant cell arteritis (according to the American College of Rheumatology classification criteria) diagnosed between October 1999 and January 2012. Cerebrovascular accident was found at initial presentation in 6 patients (6.1 %, 95 % CIs 2.3-12.9). Most of them had other symptoms of giant cell arteritis when the disease began. Signs reflecting the involvement of vertebro-basilar territory were present in 3 cases. No other case of cerebrovascular accident was described during the follow-up of patient; particularly no case of cerebrovascular accident occurred once corticosteroid therapy for the treatment of giant cell arteritis had been initiated. No differences in the epidemiologic, clinical and laboratory features at the time of diagnosis between patients who had cerebrovascular accidents and the rest of the giant cell arteritis patients were observed. Prognosis was good in our survey. However, there was no case of bilateral vertebral artery occlusion, a condition associated with poor prognosis. The present study confirms that cerebrovascular accidents may be the initial manifestation of giant cell arteritis, an argument in favor of a direct effect of the vasculitis in the development of cerebrovascular accidents rather than a complication of the corticosteroid therapy. The diagnosis of giant cell arteritis should always be considered in an elderly patient with stroke and an unexplained elevation of inflammatory biomarkers.
Published: 2013

24. Syndrome de Cogan

Author: Thierry Zenone
Subjects: Gynecology, medicine.medical_specialty, business.industry, medicine, General Medicine, business
Abstract: Points essentiels Le syndrome Cogan est une maladie rare, de cause inconnue, caracterisee par une atteinte inflammatoire oculaire (principalement de type keratite interstitielle) et audio-vestibulaire (essentiellement surdite de perception de survenue brutale) en l’absence d’infection syphilitique. La diversite des manifestations ophtalmologiques et audio-vestibulaires observees au cours du syndrome de Cogan atypique doit rendre prudent avant de retenir ce diagnostic qui peut mimer de multiples maladies systemiques. Les corticoides restent le traitement le plus largement utilise et le plus efficace. Pour les patients necessitant des doses elevees et prolongees, un immunosuppresseur doit etre propose. Le methotrexate est l’agent de premiere ligne a visee d’epargne cortisonique. Cependant, en l’absence d’atteinte systemique ou d’atteinte ophtalmologique severe non controlee par corticoides locaux, les patients ne doivent pas etre soumis a des traitements prolonges par corticosteroides ou immunosuppresseurs, potentiellement iatrogenes, en particulier lorsque le gain d’audition est faible avec leur utilisation. L’effet des anti-TNF-alpha a ete recemment etudie. L’infliximab pourrait etre une alternative therapeutique en cas d’echec de la corticotherapie et des immunosuppresseurs. Le traitement pourrait etre plus efficace s’il etait debute a un stade precoce de la maladie, lorsque les lesions sont encore reversibles.
Published: 2013

25. A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: a EUSTAR prospective study

Author: Antonio C. Zea Mendoza, Jean Sibilia, Kamal Solanki, Cristina Mihaela Tanaseanu, Fredrick M. Wigley, Guido Valesini, M. Govoni, Lisa K. Stamp, Christopher P. Denton, Yolanda Braun-Moscovici, Ruxandra Ionescu, Øyvind Midtvedt, Ileana Nicoara, Aleksandra Stanković, Rüdiger Hein, Alina Dumitrascu, Susanne Ullman, Alan Tyndall, Sergio A. Jimenez, Irena Butrimiene, Alan Doube, Eugene J. Kucharz, Mohammed Tikly, Pier Luigi Meroni, Simon Stebbings, Renata Sokolik, Alexandra Balbir Gurman, Roger Hesselstrand, Kirsten Damgaard, Francesco Paolo Cantatore, Razvan Ionitescu, Silvana Zeni, Marco Matucci-Cerinic, Maria Rosa Pozzi, Jacques-Eric Gottenberg, Srdan Novak, Ana Maria Gherghe, David Launay, Liliana Groppa, Carlomaurizio Montecucco, Roxana Sfrent Cornateanu, Stefan Heitmann, Paloma García de la Peña Lefebvre, Daniela Opris, Peter T. Chapman, Line V. Iversen, Bernard Coleiro, Ulf Müller-Ladner, John Highton, Mara Oleszowsky, Gabriella Szücs, Magdalena Kopec-Medrek, Carlos De La Puente Buijdos, Paola Caramaschi, Magdalena Szmyrka-Kaczmarek, Rucsandra Dobrota, Gabriele Valentini, Fabiana Montoya, Blaz Rozman, Alberto Sulli, Hélène Chifflot, Raffaella Scorza, Patricia Carreira, Paulius Venalis, Lisa Maria Bambara, Torhild Garen, Isabela Tiglea, Agneta Scheja, Duska Martinovic, Jörg H W Distler, Jörg Henes, Giovanni Lapadula, Luc Mouthon, Diana Karpec, Douglas J. Veale, Valeria Riccieri, Nicolas Hunzelmann, Muriel Elhai, Serena Guiducci, Codrina Ancuta, Simonetta Pisarri, Thierry Zenone, Esthela Loyo, Branimir Anić, Claudia Günther, R. Becvar, Eugen Russu, Serena Vettori, Carlo Chizzolini, Vanessa Smith, Mengtao Li, Stanislaw Sierakowsky, Carmel Mallia, Małgorzata Widuchowska, Carolina de Souza Müller, László Czirják, Algirdas Venalis, Adrian Hij, Marta Valero Exposito, Simona Rednic, Miroslav Mayer, Laura Groseanu, Walter Alberto Sifuentes Giraldo, Murray Baron, Dominique Farge, Anna Kotulska, Marko Baresic, Svetlana Agachi, Martin Aringer, Merete Engelhart, John L. O'Donnell, Jérôme Avouac, Filip De Keyser, Ulrich A. Walker, Roberto Caporali, Harald Burkhardt, P. G. Vlachoyiannopoulos, Maurizio Cutolo, Frank A. Wollheim, Edoardo Rosato, Suzanne Kafaja, Valderílio Feijó Azevedo, Kilian Eyerich, Paolo Airò, Emmanuel Chatelus, L. Ananieva, Ira Litinsky, Andrea Lo Monaco, Vanesa Cosentino, Rita Rugiene, Eric Hachulla, P. Saar, Bojana Stamenkovic, Brigitte Krummel-Lorenz, Yannick Allanore, Elisabeth Knott, Oliver Distler, Matthias Seidel, Silvia Rodriguez Rubio, Franco Cozzi, Mihai Bojinca, Nemanja Damjanov, Maria João Salvador, Joanna Busquets, Otylia Kowal Bielecka, André Kahan, Jacek Szechiński, Daniel E. Furst, C. Mihai, Rodica Chirieac, Ewa Morgiel, Georg Schett, Armando Gabrielli, Giovanna Cuomo, Piotr Wiland, Maya N. Starovoytova, Sebastião Cezar Radominski, Gitte Strauss, Lealea Chiaburu, Florenzo Iannone, Carol M. Black, Andrea Himsel, Eduardo Kerzberg, Cecília Varjú, Vera Ortiz Santamaria, Gabriela Riemekasten, Dorota Krasowska, Marilena Gorga, Monica Popescu, Marie O'Rourke, Henrik Nielsen, Raffaele Pellerito, Ada Corrado, Elhai, M, Avouac, J, Walker, Ua, Matucci Cerinic, M, Riemekasten, G, Airò, P, Hachulla, E, Valentini, Gabriele, Carreira, Pe, Cozzi, F, Balbir Gurman, A, Braun Moscovici, Y, Damjanov, N, Ananieva, Lp, Scorza, R, Jimenez, S, Busquets, J, Li, M, Müller Ladner, U, Kahan, A, Distler, O, Allanore, Y, EUSTAR co, Author, EUSTAR co, Authors, Matucci-Cerinic, M, Airo, P, Valentini, G, Gurman, Ab, Braun-Moscovici, Y, Mt, Li, Muller-Ladner, U, Allanore, Y EUSTAR co-authors: Serena Guiducci, Alan, Tyndall, Giovanni, Lapadula, Florenzo, Iannone, Radim, Becvar, Stanislaw, Sierakowsky, Otylia Kowal Bielecka, Maurizio, Cutolo, Alberto, Sulli, Cuomo, Giovanna, Vettori, Serena, Simona, Rednic, Ileana, Nicoara, Vlachoyiannopoulos, P, Montecucco, C, Roberto, Caporali, Srdan, Novak, László, Czirják, Cecilia, Varju, Carlo, Chizzolini, Eugene, J Kucharz, Anna, Kotulska, Magdalena, Kopec-Medrek, Malgorzata, Widuchowska, Blaz, Rozman, Carmel, Mallia, Bernard, Coleiro, Armando, Gabrielli, Dominique, Farge, Adrian, Hij, Roger, Hesselstrand, Agneta, Scheja, Frank, Wollheim, Duska, Martinovic, Govoni, M, Andrea Lo Monaco, Nicolas, Hunzelmann, Raffaele, Pellerito, Lisa Maria Bambara, Paola, Caramaschi, Carol, Black, Christopher, Denton, Jörg, Hene, Vera Ortiz Santamaria, Stefan, Heitmann, Dorota, Krasowska, Matthias, Seidel, Mara, Oleszowsky, Harald, Burkhardt, Andrea, Himsel, Maria, J Salvador, Bojana, Stamenkovic, Aleksandra, Stankovic, Mohammed, Tikly, Maya, N Starovoytova, Merete, Engelhart, Gitte, Strau, Henrik, Nielsen, Kirsten, Damgaard, Gabriella, Szüc, Antonio Zea Mendoza, Carlos de la Puente Buijdos, Walter, A Sifuentes Giraldo, Øyvind, Midtvedt, Torhild, Garen, David, Launay, Guido, Valesini, Valeria, Riccieri, Ruxandra Maria Ionescu, Daniela, Opri, Laura, Groseanu, Fredrick, M Wigley, Carmen, M Mihai, Roxana Sfrent Cornateanu, Razvan, Ionitescu, Ana Maria Gherghe, Marilena, Gorga, Rucsandra, Dobrota, Mihai, Bojinca, Georg, Schett, Jörg Hw Distler, Pierluigi, Meroni, Silvana, Zeni, Luc, Mouthon, Filip De Keyser, Vanessa, Smith, Francesco, P Cantatore, Ada, Corrado, Susanne, Ullman, Line, Iversen, Maria, R Pozzi, Kilian, Eyerich, Rüdiger, Hein, Elisabeth, Knott, Jacek, Szechinski, Piotr, Wiland, Magdalena, Szmyrka-Kaczmarek, Renata, Sokolik, Ewa, Morgiel, Brigitte, Krummel-Lorenz, Petra, Saar, Martin, Aringer, Claudia, Günther, Branimir, Anic, Marko, Baresic, Miroslav, Mayer, Sebastião, C Radominski, Carolina de Souza Müller, Valderílio, F Azevedo, Svetlana, Agachi, Liliana, Groppa, Lealea, Chiaburu, Eugen, Russu, Thierry, Zenone, Simon, Stebbing, John, Highton, Lisa, Stamp, Peter, Chapman, Murray, Baron, John, O'Donnell, Kamal, Solanki, Alan, Doube, Douglas, Veale, Marie, O'Rourke, Esthela, Loyo, Edoardo, Rosato, Simonetta, Pisarri, Cristina-Mihaela, Tanaseanu, Monica, Popescu, Alina, Dumitrascu, Isabela, Tiglea, Rodica, Chirieac, Codrina, Ancuta, Daniel, E Furst, Suzanne, Kafaja, Paloma García de la Peña Lefebvre, Silvia Rodriguez Rubio, Marta Valero Exposito, Jean, Sibilia, Emmanuel, Chatelu, Jacques Eric Gottenberg, Hélène, Chifflot, Ira, Litinsky, Algirdas, Venali, Irena, Butrimiene, Paulius, Venali, Rita, Rugiene, Diana, Karpec, Eduardo, Kerzberg, Fabiana, Montoya, Vanesa, Cosentino, and Chizzolini, Carlo
Subjects: 0301 basic medicine, Male, heart dysfunction, Databases, Factual, Epidemiology, autoimmune diseases, epidemiology, systemic sclerosis, Kaplan-Meier Estimate, 0302 clinical medicine, Cardiovascular Disease, Immunology and Allergy, Prospective Studies, Age of Onset, skin and connective tissue diseases, Prospective cohort study, ddc:616, Orvostudományok, Middle Aged, Prognosis, Connective tissue disease, 3. Good health, Europe, Cardiovascular Diseases, Cohort, Disease Progression, Female, Autoimmune Diseases, Systemic Sclerosis, Human, Adult, medicine.medical_specialty, Prognosi, Immunology, Socio-culturale, Klinikai orvostudományok, Autoimmune Disease, General Biochemistry, Genetics and Molecular Biology, Follow-Up Studie, 03 medical and health sciences, Sex Factors, Rheumatology, Internal medicine, medicine, Humans, Sex Distribution, Systemic Sclerosi, Aged, 030203 arthritis & rheumatology, Lupus erythematosus, Scleroderma, Systemic, business.industry, medicine.disease, Pulmonary hypertension, Prospective Studie, 030104 developmental biology, Heart failure, Age of onset, business, Follow-Up Studies
Abstract: OBJECTIVES: In agreement with other autoimmune diseases, systemic sclerosis (SSc) is associated with a strong sex bias. However, unlike lupus, the effects of sex on disease phenotype and prognosis are poorly known. Therefore, we aimed to determine sex effects on outcomes. METHOD: We performed a prospective observational study using the latest 2013 data extract from the EULAR scleroderma trials and research (EUSTAR) cohort. We looked at (i) sex influence on disease characteristics at baseline and (ii) then focused on patients with at least 2 years of follow-up to estimate the effects of sex on disease progression and survival. RESULTS: 9182 patients with SSc were available (1321 men) for the baseline analyses. In multivariate analysis, male sex was independently associated with a higher risk of diffuse cutaneous subtype (OR: 1.68, (1.45 to 1.94); p
Published: 2014

26. Camptocormia as a clinical manifestation of polymyositis/systemic sclerosis overlap myositis associated with anti-Ku

Author: Nathalie Streichenberger, Marie Puget, and Thierry Zenone
Subjects: Male, Pathology, medicine.medical_specialty, Movement disorders, Immunology, Polymyositis, Spinal Curvatures, Scleroderma, Muscular Atrophy, Spinal, Camptocormia, Rheumatology, Internal medicine, medicine, Humans, Immunology and Allergy, Ku Autoantigen, Myositis, Aged, Autoantibodies, Scleroderma, Systemic, business.industry, Antigens, Nuclear, Sclerodactyly, medicine.disease, DNA-Binding Proteins, medicine.symptom, business, Rhabdomyolysis
Abstract: Camptocormia, an abnormal truncal flexion posture that occurs while walking or standing, is usually caused by various hypokinetic movement disorders, mainly Parkinson disease. We describe the case of a man with subacute onset of camptocormia. Quadriceps muscle biopsy showed significant rhabdomyolysis, few isolated inflammatory cells and mild expression of type I MHC in few fibers, a pattern usually found in immune-mediated necrotizing myopathies. Myositis was associated with Raynaud's phenomenon, mild sclerodactyly, and anti-Ku antibodies leading to the diagnosis of polymyositis/systemic sclerosis overlap myositis. The posture showed modest improvement in response to treatment.
Published: 2012

27. SystemicBartonella henselaeInfection in Immunocompetent Adult Presenting as Fever of Unknown Origin

Author: Thierry Zenone
Subjects: Bartonella henselae, biology, business.industry, lcsh:R, lcsh:Medicine, Case Report, Cat-scratch disease, General Medicine, medicine.disease, biology.organism_classification, Serology, Immunology, medicine, Fever of unknown origin, business
Abstract: Systemic clinical presentations of infection caused byBartonella henselaeare rare in immunocompetent adults. We report four cases with hepatic and/or splenic involvement, presenting as fever of unknown origin. We discuss diagnosis and treatment of this infection.Bartonella henselaeserology allows an easy diagnosis of hepatosplenic involvement in cat scratch disease, a clinical picture that appears to be underrecognized.
Published: 2011

28. Syndrome de Cogan : étude rétrospective nationale française

Author: M. Hamidou, Olivier Fain, Thomas Quemeneur, C. Durtette, Bertrand Lioger, Thierry Zenone, Christophe Deligny, Robin Dhote, Pascal Sève, Arsène Mekinian, Jean-Emmanuel Kahn, Eric Hachulla, Edouard Pertuiset, P.Y. Hatron, Anne Grasland, Christian Lavigne, M. Resche-Rigon, B. de Wazières, T. Le Gallou, Guillaume Gondran, J. Pouchot, Marc Lambert, Thomas Papo, and Cédric Landron
Subjects: Gastroenterology, Internal Medicine
Abstract: Introduction Le syndrome de Cogan est une pathologie rare dont la prise en charge reste mal codifiee. L’objectif de l’etude etait de determiner l’efficacite de l’infliximab en comparaison aux DMARDs et la corticotherapie seule. Patients et methodes Nous avons mene une etude retrospective francaise nationale incluant des patients atteints du syndrome de Cogan (n = 40) et une revue de la litterature des cas (n = 22) en analysant l’efficacite des DMARDs et des anti-TNFa, en particulier l’infliximab. Les donnees etaient analysees en considerant les differentes lignes de traitements poolees pour tous les patients. Resultats Nous avons inclus 62 patients (31 femmes) (âge median de 37 ans [2–76]). Au moment du diagnostic, 61 patients (98 %) avaient des symptomes vestibulo-auditifs, avec une surdite bilaterale dans 41 %. Les signes oculaires etaient presents chez 57 patients (92 %), avec une keratite interstitielle dans le 31 (51 %). Le traitement de premiere ligne se composait de corticoides seuls (n = 43 ; 70 %) ou associes a d’autres immunosuppresseurs (n = 18 ; 30 %). Une reponse vestibulo-auditive etait note dans 32/39 (82 %) et 15/19 (79 %) avec corticoides seuls et associes a des immunosuppresseurs, une reponse oculaire dans 23/28 (82 %) et 10/14 (71 %) et une reponse systemique dans 13/43 (30 %) et 4/18 (22 %), respectivement (p = 0,8). Soixante et un patients avaient recu au total 126 lignes de traitement, consistant en corticoides seuls (n = 51 lignes), avec DMARDs (n = 65) et l’infliximab (n = 10). Une reponse vestibulo-auditive etait significativement plus frequente avec l’infliximab que DMARDs ou corticoides seuls (80 % contre 39 % et 35 %, respectivement), tandis que les taux de reponse systemique et oculaire etaient similaires. L’infliximab etait le seul facteur predictif d’amelioration vestibulo-auditif (odd 20,7 [intervalle de confiance a 95 % : 1,65 ; 260], p = 0,019). Conclusion Infliximab pourrait induire une reponse vestibulo-auditive a la difference des DMARDs dans le syndrome de Cogan, mais des etudes prospectives sont necessaires.
Published: 2017

29. Fever of Unknown Origin in Rheumatic Diseases

Author: Thierry Zenone
Subjects: Vasculitis, Microbiology (medical), Pathology, medicine.medical_specialty, business.industry, Connective tissue, Disease, Granulomatous Disease, Chronic, medicine.disease, Fever of Unknown Origin, Polymyalgia rheumatica, Giant cell arteritis, Infectious Diseases, medicine.anatomical_structure, Granulomatous disease, Rheumatic Diseases, Humans, Medicine, Fever of unknown origin, Connective Tissue Diseases, business
Abstract: Noninfectious inflammatory diseases (connective tissue diseases, vasculitis syndromes, granulomatous diseases) emerged as the most frequent cause of fever of unknown origin in western countries. Among these diseases, giant cell arteritis and polymyalgia rheumatica are the most frequent specific diagnosis in the elderly and adult-onset Still's disease the most frequent in younger patients. This article focuses on noninfectious inflammatory diseases as a cause of classic fever of unknown origin (mainly rheumatic diseases, such as vasculitis and connective tissue diseases).
Published: 2007

30. A comparison between nailfold capillaroscopy patterns in adulthood in juvenile and adult-onset systemic sclerosis: A EUSTAR exploratory study

Author: F. Lauffer, Kati Otsa, Oliver Distler, S. Zeni, Marco Matucci Cerinic, Maria Rosa Pozzi, Margarita Pileckyte, John Highton, Paola Caramaschi, Jacek Szechiński, Maria João Salvador, Diana Karpec, Maurizio Cutolo, Codrina Ancuta, Patrizia Boracchi, Simonetta Pisarri, Fabiana Montoya, Vanessa Smith, Mengtao Li, Carolina de Souza Müller, Patricia Carreira, C. Mihai, Henrik Nielsen, Luc Mouthon, L. Denisov, Marc Frerix, Pier Luigi Meroni, Øyvind Midtvedt, Francesco Paolo Cantatore, Ada Corrado, Sebastião Cezar Radominski, Serena Guiducci, Francesco Puppo, Simon Stebbings, Armando Gabrielli, Giovanna Cuomo, Irena Butrimiene, Piotr Wiland, Ira Litinsky, Maria Uprus, Merete Engelhart, Roger Hesselstrand, Ulrich A Walker, Rodica Chirieac, Ulf Müller-Ladner, David Launay, Kirsten Damgaard, Kamal Solanki, Cristina Mihaela Tanaseanu, Torhild Garen, Isabela Tiglea, Aleksandra Stanković, L. Ananieva, Francesca Ingegnoli, Magdalena Szmyrka-Kaczmarek, Jörg Henes, Alan Tyndall, Roberta Gualtierotti, Rüdiger Hein, Ewa Morgiel, Edoardo Rosato, Ivan Foeldvari, Valderílio Feijó Azevedo, Gitte Strauss, Valeria Riccieri, Anna Kotulska, Marta Valero Exposito, R. Becvar, José António Pereira da Silva, Blaz Rozman, Vera Ortiz-Santamaria, Paloma García de la Peña Lefebvre, Szilvia Szamosi, Małgorzata Widuchowska, Gabriella Szücs, Martin Aringer, Paulius Venalis, Roberto Caporali, Kilian Eyerich, Florenzo Iannone, Alina Dumitrascu, Eugene J. Kucharz, Laura Groseanu, Alessandra Vacca, Monica Popescu, Cristiane Kayser, Yannick Allanore, Brigitte Krummel-Lorenz, P. Saar, Mihai Bojinca, Magdalena Kopec-Medrek, Eduardo Kerzberg, Cecília Varjú, Nemanja Damjanov, Luis Eduardo Coelho Andrade, Rita Rugiene, Paolo Airò, Filip De Keyser, Nicola Ughi, Bojana Stamenkovic, Claudia Günther, Ruxandra Ionescu, László Czirják, Matthias Seidel, Silvia Rodriguez Rubio, Paola Gottschalk, Dirk M. Wuttge, Alan Doube, Vanesa Cosentino, Thierry Zenone, Dominique Farge-Bancel, Esthela Loyo, Algirdas Venalis, Renata Sokolik, Alberto Sulli, Rosario Foti, Stefan Heitmann, Eric Hachulla, Juan José Alegre-Sancho, Carlomaurizio Montecucco, Daniela Opris, Ingegnoli, F, Boracchi, P, Gualtierotti, R, Smith, V, Cutolo, M, Foeldvari, I, Airò, P, Alegre-Sancho, Jj, Allanore, Y, Ananieva, Lp, Ancuta, C, Andrade, Le, Aringer, M, Becvar, R, Bojinca, M, Butrimiene, I, Cantatore, Fp, Caporali, R, Caramaschi, P, Carreira, Pe, Chirieac, R, Corrado, A, Cosentino, V, Cuomo, G, Czirjak, L, Da Silva, Ja, la Peña Lefebvre, Pg, De Keyser, F, de Souza Müller, C, Damgaard, K, Damjanov, N, Denisov, Ln, Distler, O, Doube, A, Dumitrascu, A, Engelhart, M, Exposito, Mv, Eyerich, K, Farge-Bancel, D, Azevedo, Vf, Foti, R, Frerix, M, Gabrielli, A, Garen, T, Gottschalk, P, Groseanu, L, Guiducci, S, Günther, C, Hachulla, Hein, R, Heitmann, S, Henes, J, Hesselstrand, R, Highton, J, Iannone, F, Ionescu, Rm, Kayser, C, Karpec, D, Kerzberg, E, Kotulska, A, Kopec-Medrek, M, Kucharz, E, Krummel-Lorenz, B, Lauffer, F, Launay, D, Li, M, Litinsky, I, Loyo, E, Cerinic, Mm, Meroni, P, Midtvedt, Ø, Mihai, Cm, Montecucco, C, Montoya, F, Morgiel, E, Mouthon, L, Müller-Ladner, U, Nielsen, H, Opris, D, Ortiz-Santamaria, V, Otsa, K, Pileckyte, M, Pisarri, S, Popescu, M, Pozzi, Mr, Puppo, F, Radominski, Sc, Riccieri, V, Rosato, E, Rozman, B, Rubio, Sr, Rugiene, R, Saar, P, Salvador, Mj, Seidel, M, Sokolik, R, Solanki, K, Stamenkovic, B, Stankovic, A, Stebbings, S, Strauss, G, Sulli, A, Szamosi, S, Szechinski, J, Szmyrka-Kaczmarek, M, Szücs, G, Tanaseanu, Cm, Tiglea, I, Tyndall, A, Ughi, N, Uprus, M, Vacca, A, Varju, C, Venalis, A, Venalis, P, Walker, Ua, Widuchowska, M, Wiland, P, Wuttge, Dm, Zeni, S, and Zenone, T.
Subjects: Adult, Male, medicine.medical_specialty, Pathology, Adolescent, Klinikai orvostudományok, Biochemistry, Juvenile systemic sclerosi, Scleroderma, Microscopic Angioscopy, Systemic sclerosi, Scleroderma, Localized, Young Adult, Medicine, Juvenile, Humans, Young adult, Age of Onset, skin and connective tissue diseases, Child, Nailfold Capillaroscopy, Videocapillaroscopy, Aged, Retrospective Studies, EUSTAR, Scleroderma, Systemic, integumentary system, Capillaroscopy, business.industry, Similar distribution, Microcirculation, Autoantibody, Retrospective cohort study, Orvostudományok, Cell Biology, Middle Aged, medicine.disease, Dermatology, Capillaries, Nailfold capillaroscopy, Female, Age of onset, Cardiology and Cardiovascular Medicine, business, Juvenile systemic sclerosis, Systemic sclerosis
Abstract: Objective: Qualitative capillaroscopy patterns in juvenile- and adult-onset systemic sclerosis (SSc) were studied in adulthood using data from the EULAR Scleroderma Trials and Research (EUSTAR) database. Methods: Data collected between June 2004 and April 2013 were examined with focus on capillaroscopy. In this retrospective exploratory study, series of patients with juvenile-onset SSc were matched with series of adult-onset SSc having the same gender and autoantibody profile. Results: 30 of 123 patients with juvenile-onset and 2108 of 7133 with adult-onset SSc had data on capillaroscopy. Juvenile-onset SSc showed scleroderma pattern more frequently than adult-onset SSc (93.3% and 88%). The OR was 2.44 and 95% Cl 0.57-10.41. An active scleroderma pattern was present in 58% of juvenile- and 61% of adult-onset SSc. The OR was 0.91 and 95% Cl 0.28-2.93. The late scleroderma pattern was present in 61% of juvenile- and 55.5% of adult-onset SSc. The OR was 1.06 and 95% Cl 0.34-3.56. Conclusion: This is the first exploratory study on the comparison of capillaroscopy between juvenile- and adult-onset SSc in adulthood. Juvenile-onset SSc had an increase prevalence of sderoderma pattern, but a similar distribution of the three patterns was suggested. Further studies are needed to define this issue. (C) 2015 Elsevier Inc. All rights reserved.
Published: 2015

31. The Clinical Spectrum and Therapeutic Management of Hypocomplementemic Urticarial Vasculitis: Data From a French Nationwide Study of Fifty‐Seven Patients

Author: Selim Aractingi, Denis Jullien, Antoine Petit, François Maurier, Isabelle Guichard, Leonardo Astudillo, Pascal Godmer, Béatrice Flageul, Luc Mouthon, Clotilde Martel, Martine Bagot, Véronique Frémeaux-Bacchi, Florence Cordoliani, Adrien Bigot, Géraldine Jeudy, Noémie Jourde-Chiche, Didier Bessis, Benjamin Terrier, Alban Deroux, Mikael Ebbo, Sara Melboucy-Belkhir, Nicolas Dupin, Bernard Simorre, C. Morice, Laurent Machet, Emmanuel Héron, Marie Jachiet, C. Abasq, Alain Le Quellec, Marie-Sylvie Doutre, P. Belenotti, Christian Lavigne, Marie-Alice Macher, Loïc Guillevin, Laurence Bouillet, Thierry Zenone, Eric Hachulla, CHU Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Université Paris Descartes - Paris 5 (UPD5), Hôpital Saint-Louis, Université Paris Diderot - Paris 7 (UPD7)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Centre Hospitalier Universitaire [Grenoble] (CHU), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Cellules Souches, Plasticité Cellulaire, Médecine Régénératrice et Immunothérapies (IRMB), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), Centre hospitalier régional Metz-Thionville (CHR Metz-Thionville), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), CHU Toulouse [Toulouse], Hôpital de la Conception [CHU - APHM] (LA CONCEPTION ), CHU Trousseau [Tours], Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), CHU Bordeaux [Bordeaux], Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne), Université Lille Nord de France (COMUE), CIC Quinze-Vingts, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, Service de Dermatologie (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), CHU Marseille, Hôpital Edouard Herriot [CHU - HCL], Hospices Civils de Lyon (HCL), Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), AP-HP Hôpital universitaire Robert-Debré [Paris], CHU Limoges, Hôpital Jean Verdier [Bondy], CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Hopital Saint-Louis, Assistance Publique – Hôpitaux de Paris (AP-HP), Centre hospitalier de Valence, Physiopathologie vasculaire : interactions cellulaires, signalisation et vieillissement, Université Joseph Fourier - Grenoble 1 (UJF)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital Européen Georges Pompidou [APHP] (HEGP), Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Service de médecine interne et centre de référence des maladies rares [CHU Cochin], CHU Cochin [AP-HP]-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Hôpital de la Conception [CHU - APHM] (LA CONCEPTION), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts (CHNO), Hôpital Jean Verdier [AP-HP], Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Université de Montpellier (UM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)
Subjects: medicine.medical_specialty, Constitutional symptoms, Immunology, Azathioprine, 030207 dermatology & venereal diseases, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, medicine, Immunology and Allergy, Livedo reticularis, 030203 arthritis & rheumatology, Angioedema, business.industry, Hydroxychloroquine, medicine.disease, Dermatology, 3. Good health, Surgery, [SDV.MHEP.RSOA]Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, Etiology, Rituximab, medicine.symptom, business, Vasculitis, medicine.drug
Abstract: International audience; OBJECTIVE:Hypocomplementemic urticarial vasculitis (HUV) is an uncommon vasculitis of unknown etiology that is rarely described in the literature. We undertook this study to analyze the clinical spectrum and the therapeutic management of patients with HUV.METHODS:We conducted a French nationwide retrospective study that included 57 patients with chronic urticaria, histologic leukocytoclastic vasculitis, and hypocomplementemia. We assessed clinical and laboratory data and evaluated the patients' cutaneous and immunologic responses to therapy. We evaluated treatment efficacy by measuring the time to treatment failure.RESULTS:Urticarial lesions were typically more pruritic than painful and were associated with angioedema in 51% of patients, purpura in 35%, and livedo reticularis in 14%. Extracutaneous manifestations included constitutional symptoms (in 56% of patients) as well as musculoskeletal involvement (in 82%), ocular involvement (in 56%), pulmonary involvement (in 19%), gastrointestinal involvement (in 18%), and kidney involvement (in 14%). Patients with HUV typically presented with low C1q levels and normal C1 inhibitor levels, in association with anti-C1q antibodies in 55% of patients. Hydroxychloroquine or colchicine seemed to be as effective as corticosteroids as first-line therapy. In patients with relapsing and/or refractory disease, rates of cutaneous and immunologic response to therapy seemed to be higher with conventional immunosuppressive agents, in particular, azathioprine, mycophenolate mofetil, or cyclophosphamide, while a rituximab-based regimen tended to have higher efficacy. Finally, a cutaneous response to therapy was strongly associated with an immunologic response to therapy.CONCLUSION:HUV represents an uncommon systemic and relapsing vasculitis with various manifestations, mainly, musculoskeletal and ocular involvement associated with anti-C1q antibodies, which were found in approximately half of the patients. The best strategy for treating HUV has yet to be defined.
Published: 2015

32. Sensitivity of clinically abnormal temporal artery in giant cell arteritis

Author: Thierry Zenone and Marie Puget
Subjects: Male, medicine.medical_specialty, Biopsy, Giant Cell Arteritis, Pulsatile flow, Blood Sedimentation, Palpation, Rheumatology, Predictive Value of Tests, medicine, Humans, Sensitivity (control systems), Aged, Retrospective Studies, Aged, 80 and over, medicine.diagnostic_test, business.industry, Retrospective cohort study, medicine.disease, Temporal Arteries, Giant cell arteritis, Cerebrovascular Circulation, Pulsatile Flow, Predictive value of tests, Female, Temporal artery, Radiology, business
Published: 2013

33. Paragangliome avec syndrome inflammatoire biologique isolé : utilité de la tomographie par émission de positons

Author: Éric Zenou, Thierry Zenone, and Isabelle Morelec
Subjects: business.industry, Medicine, General Medicine, business, Nuclear medicine
Published: 2011

34. Predictors of early relapse in patients with non-infectious mixed cryoglobulinemia vasculitis: Results from the French nationwide CryoVas survey

Author: Jean-Marc Léger, Fabrice Carrat, P. Belenotti, Luc de Saint-Martin, Isabelle Marie, P. Rullier, Franck Bridoux, Guillaume Le Guenno, Thierry Zenone, Olivier Hermine, Xavier Mariette, Emmanuelle Plaisier, Benjamin Terrier, Antoine Huart, Thomas Quemeneur, Patrice Cacoub, Adeline Lacraz, Fabrice Bonnet, Olivier Hinschberger, Estibaliz Lazaro, Jean-Emmanuel Kahn, Elisabeth Diot, Patricia Senet, David Launay, Groupe d'Etude de la Thrombose de Bretagne Occidentale (GETBO), Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO)-Université de Brest (UBO)-Université de Brest (UBO), Service de néphrologie - hémodialyse et transplantation rénale, Centre hospitalier universitaire de Poitiers (CHU Poitiers), Contrôle de la Réponse Immune B et des Lymphoproliférations (CRIBL), Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503)-Centre National de la Recherche Scientifique (CNRS), Université de Brest (UBO)-Institut Brestois Santé Agro Matière (IBSAM), and Université de Brest (UBO)
Subjects: Vasculitis, medicine.medical_specialty, Cyclophosphamide, Immunology, Early Relapse, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, Recurrence, Internal medicine, medicine, Humans, Immunology and Allergy, In patient, 030304 developmental biology, 030203 arthritis & rheumatology, 0303 health sciences, Gastrointestinal tract, business.industry, Remission Induction, medicine.disease, 3. Good health, Purpura, Cryoglobulinemia, Mixed cryoglobulinemia, [SDV.IMM]Life Sciences [q-bio]/Immunology, Rituximab, France, medicine.symptom, business, Follow-Up Studies, medicine.drug
Abstract: Objective Although in most patients induction therapy leads to complete or partial remission, relapses in patients with non-infectious mixed cryoglobulinemia vasculitis (CryoVas) remain a major problem. We aimed to identify predictors of early relapses occurring within the first 12 months of treatment in such patients. Methods Patients included in the French CryoVas survey exhibiting complete/partial clinical remission and followed-up for at least 12 months after induction therapy (n = 145) were analyzed for predictors of early relapses. Results Forty out of 145 patients (28%) experienced early relapse. Relapses occurred after a median time of 9.5 months after induction therapy (3–12) and involved skin (75%), joints and peripheral nerve (28% each), kidneys (25%) and gastrointestinal tract (5%). Baseline factors associated with an early relapse were purpura [HR 3.35 (1.02–10.97), P = 0.046], cutaneous necrosis [HR 4.46 (1.58–12.57), P = 0.005] and articular involvement [HR 2.20 (1.00–4.78), P = 0.048]. The only factor negatively associated with an early relapse during follow-up was the achievement of complete immunological response [HR 0.07 (0.01–0.51), P = 0.009]. The use of corticosteroids plus rituximab or cyclophosphamide tended to be associated negatively with early relapse [HR 0.43 (0.17–1.08), P = 0.07]. Conclusion In patients with non-infectious CryoVas, main predictors of early relapses after initial remission are purpura, articular involvement, and cutaneous necrosis. The absence of complete immunological response during follow-up was associated with early relapse. These findings may help in adapting future treatment strategies.
Published: 2014

35. Pericardial effusion and giant cell arteritis

Author: Marie Puget and Thierry Zenone
Subjects: Male, medicine.medical_specialty, Immunology, Giant Cell Arteritis, Asymptomatic, Pericardial effusion, Pericardial Effusion, Pericarditis, Rheumatology, Internal medicine, medicine, Immunology and Allergy, Humans, Aortitis, Aged, Retrospective Studies, Aged, 80 and over, business.industry, Retrospective cohort study, Middle Aged, medicine.disease, Prognosis, Surgery, Giant cell arteritis, Female, medicine.symptom, Vasculitis, business
Abstract: Pericardial effusion in patients with giant cell arteritis has been described in case reports. The exact prevalence of this manifestation is unknown. The objective of this study was to determine the prevalence and characteristics of pericardial effusion in patients with giant cell arteritis. Retrospective data were collected from 114 patients at a single hospital with giant cell arteritis diagnosed between October 1999 and July 2013. Pericardial effusion was found in four patients (3.5 %, 95 % confidence intervals 1–8.7). In three cases, pericardial effusion was present at initial presentation and was asymptomatic. In one case, pericardial effusion was associated with aortitis and diagnosed at 6 months on thoracic CT scan because of relapse of the disease when corticosteroid was reduced. Prognosis was good in all cases. The present study confirms that pericardial effusion could occur in patients with giant cell arteritis. However, pericardial effusion is a rare manifestation of giant cell arteritis. Clinical course of pericardial effusion in the setting of giant cell arteritis is usually benign and rarely symptomatic. Recognition of this manifestation may contribute to early diagnosis of the disease.
Published: 2013

36. Efficacy of Infliximab in Cogan’s Syndrome

Author: Marie Couret, Rawan Ghadban, and Thierry Zenone
Subjects: medicine.medical_specialty, Abdominal pain, business.industry, Interstitial keratitis, Immunology, Episcleritis, medicine.disease, Dermatology, Infliximab, Keratitis, Surgery, Rheumatology, medicine, Immunology and Allergy, medicine.symptom, business, Scleritis, Aortitis, Uveitis, medicine.drug
Abstract: To the Editor: Cogan’s syndrome is a rare chronic inflammatory disease that most commonly affects adults in the third decade, with no gender predominance. Interstitial keratitis is the characteristic ocular feature, although this is not mandatory for the diagnosis; moreover, ocular manifestations such as scleritis, episcleritis, and uveitis may occur. The vestibuloauditory manifestations consist of a Meniere’s-like disease including vertigo, nausea, vomiting, tinnitus, and hearing loss. The diagnosis is made upon the association of eye disease and vestibuloauditory dysfunction, with or without systemic features (fever, asthenia, weight loss, arthritis, arthralgias, aortitis, and abdominal pain)1–4. A 48-year-old woman was admitted...
Published: 2008

37. Événements rythmiques graves au cours de la maladie de Fabry : description de 9 cas issus d’une cohorte monocentrique de 49 patients

Author: Olivier Fain, P. Charron, Jean-Marc Ziza, Laurent Aaron, J.M. Darondel, Dominique Joly, Thierry Zenone, T. Sené, J. Sebbah, and O. Lidove
Subjects: Gastroenterology, Internal Medicine
Abstract: Introduction L’hypertrophie ventriculaire gauche (HVG) est la manifestation cardiaque la plus frequente au cours de la maladie de Fabry (MF). La survenue d’evenements rythmiques graves (ERG) (arythmies ventriculaires, dysfonction sinusale, troubles de la conduction atrioventriculaire) necessitant la pose de dispositifs implantables a ete rapportee, mais la frequence, les eventuels facteurs predictifs et l’evolution de ces ERG ont peu ete etudies dans la litterature. Nous rapportons l’experience d’un centre de reference de la MF. Patients et methodes Etude retrospective monocentrique a partir d’une cohorte de patients (vivants ou decedes) avec MF affirmee par dosage enzymatique et/ou analyse genetique. Resultats Description de la cohorte. Quarante-neuf patients (19H, 30F) ont ete inclus. L’âge median au diagnostic de MF etait de 35 ans (extremes : 7–70). Le suivi median depuis le diagnostic etait de 8 ans (0–49). Vingt-sept patients (54 %) ont ete traites par traitement enzymatique substitutif (TES), introduit a un âge median de 43 ans (17–71) et pour une duree mediane de 6 ans (0–12). A la fin du suivi, l’atteinte cardiaque (HVG) etait presente chez 22 patients (44 % ; H 63 % ; F 33 %), 3 patients etaient decedes (embolie massive, sepsis severe, cancer) et 2 patients etaient perdus de vue. Description des ERG. Neuf patients (4H, 5F) ont presente un ERG dont les caracteristiques sont resumees dans l’Annexe A . Deux patients ont presente l’ERG avant le diagnostic de MF. L’âge median d’implantation etait de 53 ans (26–57) chez les hommes et de 70 ans (48–76) chez les femmes. Quatre patients etaient traites par TES au diagnostic d’ERG. Aucune mort subite d’origine cardiaque n’a ete notee. Le phenotype n’etait pas different dans les 2 groupes, avec ou sans ERG. Discussion Notre cohorte est representative de la variete phenotypique de la MF. Les ERG ont concerne 18 % des patients de cette cohorte. Les ERG surviennent de maniere plus precoce chez l’homme. Un phenotype clinique severe, une HVG sous-jacente et des troubles du rythme precedemment detectes par Holter-ECG sont frequemment retrouves dans le groupe avec ERG. La morbi-mortalite dans ce groupe est elevee. L’incidence de la pose de stimulateur cardiaque dans la MF a ete estimee 25 fois superieure a celle de la population generale. Une duree du QRS superieure a 110 ms a egalement ete associee a un risque augmente d’ERG [1] . La faiblesse de l’effectif et le caractere retrospectif de notre etude n’ont pas permis l’identification d’autres facteurs predictifs. Conclusion Compte tenu de l’incidence des ERG et du risque de mort subite, un depistage regulier par holter-ECG semble indispensable chez les patients et patientes avec MF. En complement d’une education therapeutique reguliere, nous preconisons que les patients aient constamment sur eux un ECG de reference et une carte de soins et d’urgence.
Published: 2015

38. Intérêts de la TEP-TDM au [18F]-FDG dans la dermatomyosite : fixation musculaire et activité de la maladie

Author: Aurélie Grados, G. Monseau, Olivier Lidove, B. Brihaye, F. Andry, L. Marcq, N. Martis, J.-P. Chaborel, P. Viau, D. Quinsat, J.G. Fuzibet, Thierry Zenone, and Amicale des Jeunes Internistes
Subjects: Gastroenterology, Internal Medicine
Abstract: Introduction La dermatomyosite (DM) est une myopathie inflammatoire acquise et rare, et est associee a un processus neoplasique dans environ un tiers des cas. L’utilite de la tomodensitometrie par emission de positons (TEP-TDM) au [18F]-fluorodeoxyglucose pour guider sa prise en charge initiale n’a ete que peu decrite. Nos objectifs etaient d’etudier la signification de la fixation musculaire et son interet pour l’evaluation de l’activite de la maladie. Patients et methodes Il s’agit d’une etude descriptive, retrospective et multicentrique francaise, realisee grâce a un appel a observations medie par l’Amicale des Jeunes Internistes, et colligeant des cas diagnostiques entre juin 2010 et mai 2014. L’ensemble des patients presentait les criteres diagnostiques de DM selon le consensus de l’ENMC et avait obligatoirement eu une biopsie musculaire. La TEP-TDM etait realisee dans les 3 premiers mois suivant la suspicion diagnostique de la maladie. La valeur de fixation normalisee du radiotraceur, appele standardized uptake value (SUV), etait mesuree aux niveaux du muscle le plus hyperfixant (MHF), d’un muscle long du rachis dorsal (MLD), et du parenchyme hepatique (PH). Le choix du muscle etudie etait laisse a l’appreciation du medecin nucleaire. Les SUV des muscles etaient rapportes a un meme SUVmoyen du PH ; un ratio de SUV MHF/MLD etait aussi systematiquement calcule. Resultats Quinze patients d’âge median de 60 ans (extremes, 27–85) avec un sex-ratio H/F de 0,27 etaient inclus. Aucun patient n’etait diabetique et les glycemies avant l’examen etaient normales. La duree mediane entre la suspicion diagnostique et la realisation de la TEP-TDM etait de 11 jours (IQR, 7–22). Seulement 4 patients etaient sous un traitement immunosuppresseur lors de l’examen. La duree mediane depuis l’initiation du traitement chez ces 4 patients etait de 9 jours (extremes, 6–60). Tous les patients presentaient une hyperfixation musculaire au TEP-TDM. Le rapport SUV MHF/PH etait de 2,07 (IQR, 1,35–2,8) et etait significativement superieur a celui des SUV MLD/PH (0,47 [IQR, 0,47–0,77]) (p Discussion Un lien entre SUV et la severite de la myosite a deja ete evoque dans une des rares publications sur le sujet. La TEP-TDM permet une evaluation semi-quantitative de l’inflammation musculaire mais seulement quelques strategies de mesure ont ete decrites. Nous proposons une technique de mesure reproductible et facilement realisable en pratique, puisqu’il s’agit de comparer des rapports de SUV. Dans notre serie, on trouve une tres bonne sensibilite de la TEP-TDM et une difference significative entre les valeurs de SUV de muscles pathologiques et de muscle long du rachis dorsal. Ce dernier semble etre systematiquement epargne dans la DM et la mesure de son SUV pourrait constituer une valeur de reference propre a chaque patient. Un ratio MHF sur MLD superieur a 1,60 apparait ainsi comme une valeur – seuil acceptable pour l’identification de myosite –sous reserve de signes cliniques compatibles avec une DM selon les criteres des consensus. Cette valeur etait pertinente meme chez les patients sous traitement immunosuppresseur. Contrairement a l’IRM qui detecte l’œdeme au sein du muscle, la TEP-TDM localise plus precisement les foyers hypermetaboliques et offre une meilleure rentabilite dans le guidage d’une eventuelle biopsie. La TEP-TDM, qui est limitee surtout par son cout et l’irradiation, offre en plus la possibilite de depister les manifestations extramusculaires (notamment neoplasiques) associees a la DM. La correlation entre le taux de CPK et l’hyperfixation musculaire n’etait pas significative, potentiellement a cause du faible effectif de notre cohorte. Cela reste a preciser sur une population plus importante que nous sommes en train de constituer. Conclusion Cette etude montre l’utilite d’un rapport de SUV dans l’evaluation par TEP-TDM de l’atteinte musculaire au cours de la DM. Un ratio MHF sur MLD aurait une pertinence clinique, necessitant d’etre precisee sur une population plus large. Un appariement des patients de notre serie avec des temoins ne presentant pas de pathologie musculaire est envisage.
Published: 2015

39. Manifestations neurologiques centrales au cours de la granulomatose éosinophilique avec polyangéite (Churg-Strauss) : étude rétrospective de 26 patients et revue de la littérature

Author: David Launay, H. Gil, Chahéra Khouatra, G. Blaison, François Maurier, Loïc Guillevin, Benjamin Terrier, J.L. Saraux, Pascal Cathébras, Robin Dhote, R. André, and Thierry Zenone
Subjects: Gastroenterology, Internal Medicine
Abstract: Introduction La granulomatose eosinophilique avec polyangeite (GEPA) est une vascularite systemique rare touchant les vaisseaux de petit calibre. Elle se caracterise avant tout par un asthme, une hypereosinophilie sanguine et tissulaire, et des manifestations de vascularite. Alors que l’atteinte du systeme nerveux peripherique est bien connue et se manifeste essentiellement par une mononeuropathie multiple, l’atteinte du systeme nerveux central (SNC) est tres rare et exceptionnellement decrite. Il s’agit pourtant d’une atteinte initialement associee a un mauvais pronostic dans le Five-Factor Score de 1996. L’objectif de cette etude est de decrire les atteintes du SNC au cours de la GEPA. Patients et methodes Nous avons realise une etude retrospective, observationnelle, multicentrique incluant des patients ayant une GEPA selon les criteres de l’ACR et/ou la nomenclature de Chapel Hill, et une atteinte du SNC definie par une atteinte des paires crâniennes, une atteinte encephalique ou medullaire. Nous avons egalement effectue une revue systematique de la litterature. Resultats Nous avons identifie 26 patients avec GEPA et atteinte du SNC dans le cadre de notre etude (femmes 50 %, âge moyen 57 ± 15 ans), et 62 cas issus de la revue de la litterature (femmes 52 %, âge moyen 47 ± 16 ans), correspondant a un total de 88 patients, entre 1992 et 2014. Au diagnostic de GEPA, on notait un asthme dans 97 %, une hypereosinophilie dans 98 % avec un taux median de 7500/mm 3 , des anomalies sinusiennes dans 66 %, une neuropathie peripherique dans 55 %, des infiltrats pulmonaires dans 52 %, et une infiltration eosinophilique sur la biopsie dans 46 %. Il existait une atteinte cardiaque dans 41 %, avec une myocardite dans 27 %, une pericardite dans 16 %, une fibrose endomyocardique dans 8 % et une arythmie cardiaque dans 2 %. Il existait des ANCA dans 38/71 cas (55 %), de type p-ANCA et/ou anti-myeloperoxydase. L’atteinte du SNC etait presente au diagnostic de GEPA dans 86 % des cas, tandis qu’elle precedait le diagnostic dans 2 % et qu’elle survenait apres le diagnostic dans 12 %, apres un delai median de 24 mois. Parmi les presentations neurologiques centrales les plus frequentes, on trouvait : des lesions vasculaires cerebrales ischemiques dans 46 cas (52 %) ; une baisse de l’acuite visuelle dans 28 cas (32 %), en rapport avec une nevrite retrobulbaire dans 15 cas, une occlusion de l’artere centrale de la retine dans 10 cas et une cecite corticale dans 4 cas ; des hemorragies intracerebrales a type d’hematomes intracerebraux ou d’hemorragies sous-arachnoidiennes dans 21 cas (24 %) ; et une atteinte des paires crâniennes (associee ou non a une atteinte du nerf optique) dans 18 cas (20 %). Vingt-cinq patients presentaient l’association de plusieurs de ces tableaux cliniques. Le profil des patients avec baisse de l’acuite visuelle et atteinte des paires crâniennes semblait similaire. Chez les patients avec atteinte intracerebrale, les patients avec atteinte vasculaire hemorragique, comparativement a ceux avec une atteinte cerebrale ischemique, etaient plus jeunes (43 vs 58 ans), avaient moins frequemment une atteinte cardiaque (33 % vs 46 %), avaient plus frequemment des ANCA (79 % vs 52 %), et avaient un taux d’eosinophiles median plus eleve (13 000 vs 7500/mm 3 ). Le traitement de l’atteinte cerebrale reposait sur une corticotherapie dans 99 % des cas, associee a du cyclophosphamide dans 65 %, de l’azathioprine dans 15 %, et du methotrexate dans 2 %. Sur les 81 patients avec une reponse neurologique evaluable, on notait une reponse complete dans 43 %, une reponse partielle dans 43 %, et une absence de reponse dans 14 %. Onze patients sont decedes apres un suivi moyen de 36 mois. Conclusion Les atteintes du SNC de la GEPA sont dominees par 4 tableaux neurologiques distincts : une baisse de l’acuite visuelle, une atteinte des paires crâniennes, des lesions vasculaires ischemiques, et des accidents hemorragiques. Ces atteintes doivent etre connues afin de pouvoir evoquer dans ces situations le diagnostic de GEPA de maniere precoce, les sequelles neurologiques etant frequentes.
Published: 2015

40. La douleur comme facteur prédictif indépendant des complications d’organe dans la maladie de Fabry

Author: Frédéric Barbey, Bertrand Dussol, O. Lidove, Christian Lavigne, Isabelle Marie, F. Gaches, V. Leguy, Thierry Zenone, R. Jaussaud, C. Douillard, E. Hachulla, and P. Kaminsky
Subjects: Gastroenterology, Internal Medicine
Abstract: Introduction La maladie de Fabry est une maladie herefitaire de transmission liee a l’X due a un deficit en alphagalactosidase A, entrainant l’accumulation de son substrat (globotriaosylceramide : Gb3) dans les vaisseaux, neurones et cœur. Les douleurs sont le signe fonctionnel le plus handicapant, survenant chez les enfants et les jeunes adultes, caracterisees par des sensations de chaleur aigues ou chroniques dans les extremites. Ces acroparesthesies sont souvent sous-diagnostiquees. Angiokeratomes, hypohidrose and cornee verticillee sont les autres signes precoces classiques, alors que l’hypoacousie, la cardiomyopathie hypeertrophique, l’atteinte renale et les atteintes cerebrovasculaires sont les complications principales, survenant plus tardivement. Un diagnostic precoce est crucial car plus le diagnostic est precoce, plutot sera etre prescrite l’enzymotherapie (ERT), et plus cette derniere sera efficace. Le but de cette etude etait de determiner si la presence d’acroparesthesies predisaient les complications de la maladie chez les patients MF. Materiels et methodes Chez 200 patients MF, les signes cliniques, comme acroparesthesie (ACR), angiokeratome (AGK), hypohidrose (HHD), et cornee verticillee (CV) et les complications d’organes - hypoacousie (HAC), atteinte cerebrovasculaire (CER), renale (KDN) ou cardiaque (HEA) ont ete systematiquement repertories. La methodologie statistque reposait sur des regressions logistiques en stepwise avec un p Resultats Les patients etaient 78 hommes, âges de 37,7 ± 14,3 ans et 122 femmes, âgees de 42,2 ± 16,4 ans. Les hommes etaient plus symptomatiques que les femmes pour les acroparesthesies (70,5 % vs 50,0 % ; p = 0,004) mais aussi les autres symptomes cliniques : AGK (64 % vs 27,9 % ; p Conclusion Les patients MF qui presentent ou ont presentes des douleurs aigues ou chroniques sont a plus haut risque de developper des complications d’organe. Les douleurs apparaissent comme le meilleur predicteur clinique des complication de la MF. Ces symptomes doivent donc etre reconnus le plus tot possible pour demarrer une enzymotherapie precocement.
Published: 2014

41. Caractéristiques cliniques et thérapeutiques des vascularites urticariennes hypocomplémentémiques : étude nationale multicentrique rétrospective

Author: L. Mouthon, C. Martel, Adrien Bigot, Denis Jullien, Pascal Godmer, Géraldine Jeudy, Noémie Jourde-Chiche, Christian Lavigne, E. Hachulla, M.-A. Macher, Marie-Sylvie Doutre, Alban Deroux, François Maurier, Martine Bagot, Béatrice Flageul, Antoine Petit, Laurence Bouillet, B. Simorre, C. Abasq, Thierry Zenone, Laurent Machet, Florence Cordoliani, Véronique Frémeaux-Bacchi, P. Belenotti, C. Morice, L. Guillevin, A. Le Quellec, Nicolas Dupin, Isabelle Guichard, Sara Melboucy-Belkhir, Leonardo Astudillo, Benjamin Terrier, Didier Bessis, Selim Aractingi, M. Ebbo, Emmanuel Héron, and Marie Jachiet
Subjects: Dermatology
Abstract: Introduction La vascularite urticarienne hypocomplementemique (VUH) est une entite rare, associant une urticaire recidivante, une hypocomplementemie, une vascularite leucocytoclasique et une atteinte systemique. Nous rapportons les caracteristiques cliniques et therapeutiques de patients presentant une VUH. Materiel et methode Cette etude nationale, francaise, multicentrique et transdisciplinaire a inclus 57 patients ayant une VUH diagnostiquee entre 1992 et 2013. Les criteres d’inclusions etaient la presence de lesions urticariennes recidivantes, une vascularite leucocytoclasique sur la biopsie cutanee et une hypocomplementemie definie par une baisse du C3 et/ou du C4. Resultats La VUH etait isolee (75 %) ou associee a une maladie systemique (25 %), principalement le lupus erythemateux systemique. Les lesions cutanees etaient typiquement des papules erythemateuses fixes, avec une evolution de plus de 24 h, plus souvent prurigineuses (53 %) que douloureuses, associees a un angio-œdeme (51 %), du purpura (35 %) ou un livedo (14 %). Les manifestations extracutanees incluaient une atteinte articulaire (arthralgies/arthrites, 82 %), oculaire (uveite, sclerite/episclerite, conjonctivite, 59 %), respiratoire (toux, dyspnee ou trouble ventilatoire obstructif, 22 %), digestive (16 %) et renale (14 %). Les patients avaient classiquement un C1q abaisse et un C1 inhibiteur normal, alors que les anticorps anti-C1q etaient positifs chez 55 % des patients testes. Sur le plan therapeutique, l’hydroxychloroquine (HCQ) et la colchicine avaient en premiere ligne une efficacite comparable aux corticoides. En cas d’atteinte severe ou refractaire, l’efficacite de la corticotherapie seule ou associee a des immunosuppresseurs comme l’azathioprine (AZA) ou le mycophenolate mofetil (MMF) etait comparable. Le rituximab (RTX) prescrit chez 8 patients semblait avoir une superiorite sur les reponses cutanees et immunologiques. Discussion Les VUH representent un spectre de vascularites systemiques rares et volontiers refractaires aux traitements, ayant des manifestations cliniques variees, principalement articulaires et oculaires. La baisse du C1q represente un marqueur plus specifique que la presence des anticorps antiC1q. L’HCQ et la colchicine constitueraient la premiere ligne therapeutique, alors que les corticoides, seuls ou associes a un immunosuppresseur, AZA, MMF et/ou RTX, representeraient une alternative therapeutique en cas d’atteinte severe et/ou refractaire. Conclusion Cette etude est la plus large serie decrivant les caracteristiques des patients atteints de VUH illustrant le spectre des manifestations cliniques et immunologiques ainsi que la complexite de la prise en charge therapeutique.
Published: 2014

42. Prognostic factors of survival in patients with non-infectious mixed cryoglobulinaemia vasculitis: data from 242 cases included in the CryoVas survey

Author: Isabelle Marie, Jean-Marc Léger, Estibaliz Lzaro, Xavier Mariette, Adeline Lacraz, Thomas Quemeneur, Olivier Hermine, Franck Bridoux, Olivier Hinschberger, P. Rullier, Jean-Emmanuel Kahn, Fabrice Carrat, Patrice Cacoub, Thierry Zenone, Christian Pagnoux, P. Belenotti, Fabrice Bonnet, Patricia Senet, Luc de Saint Martin, Guillaume Le Guenno, Antoine Huart, Aurélie Hummel, Evguenia Krastinova, Elisabeth Diot, Benjamin Terrier, Emmanuelle Plaisier, David Launay, Immunologie - Immunopathologie - Immunothérapeutique (I3), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de Médecine Interne, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5), Epidémiologie des maladies infectieuses et modélisation (ESIM), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de recherche en épidémiologie et santé des populations (CESP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris-Sud - Paris 11 (UP11)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Versailles Saint-Quentin-en-Yvelines (UVSQ), Service de Médecine Interne [CHU Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Physiopathologie et biothérapies des maladies inflammatoires et autoimmunes, Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Department of Internal Medicine, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de Néphrologie-Transplantation-Dialyse, CHU Bordeaux [Bordeaux]-Groupe hospitalier Pellegrin, Service de Médecine Interne (MARSEILLE - Med Int), Assistance Publique - Hôpitaux de Marseille (APHM), Département de Médecine Interne et Pneumologie [Brest] (DMIP - Brest), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Groupe d'Etude de la Thrombose de Bretagne Occidentale (GETBO), Université de Brest (UBO)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO)-Université de Brest (UBO), Département de Médecine Interne (VALENCIENNES - Med Int), CH Valenciennes, Service de Néphrologie et Immunopathologie Clinique, Centre Hospitalier Universitaire de Toulouse, PRES Université de Toulouse, Foie, métabolismes et cancer, Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), CHU Clermont-Ferrand, Laboratoire d'Immunologie (EA 2686), Université de Lille, Droit et Santé, Département de Médecine Interne (MULHOUSE - Med Int), Centre Hospitalier Emile Muller [Mulhouse] (CH E.Muller Mulhouse), Groupe Hospitalier de Territoire Haute Alsace (GHTHA)-Groupe Hospitalier de Territoire Haute Alsace (GHTHA), Service de Médecine Interne B, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Hôpital Bretonneau, Service de médecine interne et centre de référence des maladies rares [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Contrôle de la Réponse Immune B et des Lymphoproliférations (CRIBL), Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503)-Centre National de la Recherche Scientifique (CNRS), Service de néphrologie - hémodialyse et transplantation rénale, Centre hospitalier universitaire de Poitiers (CHU Poitiers), Médecine Interne (VALENCE - Med Int), Centre hospitalier de Valence, Cytokines, hématopoïèse et réponse immune (CHRI), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de Neurologie [CHU Pitié-Salpêtrière], IFR70-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Régulation de la réponse immune, infection VIH-1 et autoimmunité, Université Paris-Sud - Paris 11 (UP11)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de dermatologie et allergologie [CHU Tenon], CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Remodelage et Reparation du Tissu Renal, Service de médecine interne [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Biologie et thérapeutique des pathologies immunitaires (BTPI), Université Pierre et Marie Curie - Paris 6 (UPMC)-Centre National de la Recherche Scientifique (CNRS), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP]-Université Paris Descartes - Paris 5 (UPD5), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Médecine Interne [Rouen], Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Université de Brest (UBO), Centre Hospitalier Universitaire Estaing, CHU Estaing, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP], Service de neurologie 1 [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Pitié-Salpêtrière [APHP], Service de Dermatologie [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Tenon [APHP], Centre National de la Recherche Scientifique (CNRS)-Université Pierre et Marie Curie - Paris 6 (UPMC), Immunologie - Immunopathologie - Immunothérapeutique ( I3 ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Cochin [AP-HP]-Université Paris Descartes - Paris 5 ( UPD5 ), Epidémiologie des maladies infectieuses et modélisation ( ESIM ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Centre de recherche en épidémiologie et santé des populations ( CESP ), Université de Versailles Saint-Quentin-en-Yvelines ( UVSQ ) -Université Paris-Sud - Paris 11 ( UP11 ) -Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Université de Rouen Normandie ( UNIROUEN ), Normandie Université ( NU ) -Normandie Université ( NU ) -CHU Rouen, Normandie Université ( NU ) -Normandie Université ( NU ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Service de Médecine Interne ( MARSEILLE - Med Int ), Assistance Publique - Hôpitaux de Marseille ( APHM ), Département de Médecine Interne et Pneumologie [Brest] ( DMIP - Brest ), Centre Hospitalier Régional Universitaire de Brest ( CHRU Brest ), Groupe d'Etude de la Thrombose de Bretagne Occidentale ( GETBO ), Université de Brest ( UBO ), Département de Médecine Interne ( VALENCIENNES - Med Int ), Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Laboratoire d'Immunologie ( EA 2686 ), Département de Médecine Interne ( MULHOUSE - Med Int ), Centre Hospitalier Emile Muller [Mulhouse] ( CH E.Muller Mulhouse ), Groupe Hospitalier de Territoire Haute Alsace ( GHTHA ) -Groupe Hospitalier de Territoire Haute Alsace ( GHTHA ), Centre Hospitalier Régional Universitaire [Montpellier] ( CHRU Montpellier ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Cochin [AP-HP], Contrôle de la Réponse Immune B et des Lymphoproliférations ( CRIBL ), Université de Limoges ( UNILIM ) -Génomique, Environnement, Immunité, Santé, Thérapeutique ( GEIST FR CNRS 3503 ) -Centre National de la Recherche Scientifique ( CNRS ), Université de Poitiers-Centre hospitalier universitaire de Poitiers ( CHU Poitiers ), Médecine Interne ( VALENCE - Med Int ), Cytokines, hématopoïèse et réponse immune ( CHRI ), Université Paris Descartes - Paris 5 ( UPD5 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Pitié-Salpêtrière [APHP], Université Paris-Sud - Paris 11 ( UP11 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Tenon [APHP], Biologie et thérapeutique des pathologies immunitaires ( BTPI ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Centre National de la Recherche Scientifique ( CNRS ), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Brestois Santé Agro Matière (IBSAM), Service Médecine interne et immunopathologie clinique [CHU Toulouse], Pôle IUCT [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Université de Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO)-Université de Brest (UBO)-Université de Brest (UBO), Centre National de la Recherche Scientifique (CNRS)-Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503), and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)
Subjects: Male, MESH : Retrospective Studies, [SDV]Life Sciences [q-bio], MESH : Aged, 030232 urology & nephrology, Kaplan-Meier Estimate, medicine.disease_cause, Severity of Illness Index, 0302 clinical medicine, Epidemiology, Immunology and Allergy, MESH : Female, MESH: Data Collection, MESH: Aged, MESH : Prognosis, MESH: Middle Aged, Data Collection, Mortality rate, MESH: Vasculitis, Middle Aged, Prognosis, 3. Good health, MESH : Vasculitis, Cryoglobulinemia, Area Under Curve, Female, MESH : Severity of Illness Index, MESH : Data Collection, Vasculitis, medicine.medical_specialty, MESH: Genetic Diseases, Inborn, MESH: Cryoglobulinemia, MESH : Male, Hepatitis C virus, Immunology, Renal function, General Biochemistry, Genetics and Molecular Biology, MESH: Prognosis, MESH : Kaplan-Meier Estimate, 03 medical and health sciences, MESH : Genetic Diseases, Inborn, Rheumatology, Internal medicine, MESH: Severity of Illness Index, Severity of illness, medicine, Humans, MESH : Middle Aged, In patient, MESH: Kaplan-Meier Estimate, Aged, Retrospective Studies, 030203 arthritis & rheumatology, MESH: Humans, [ SDV ] Life Sciences [q-bio], business.industry, MESH : Humans, Genetic Diseases, Inborn, Retrospective cohort study, MESH: Retrospective Studies, medicine.disease, MESH : Cryoglobulinemia, MESH: Male, Surgery, MESH: Area Under Curve, MESH : Area Under Curve, business, MESH: Female
Abstract: International audience; BACKGROUND: Data on the prognosis of non-infectious mixed cryoglobulinaemia vasculitis (CryoVas) in the era of hepatitis C virus screening are lacking. METHODS: The French multicentre and retrospective CryoVas survey included 242 patients with non-infectious mixed CryoVas. Causes of death and prognostic factors of survival were assessed and a prognostic score was determined to predict survival at 5 years. RESULTS: After a median follow-up of 35 months, 42 patients (17%) died. Causes of death were mainly serious infections (50%) and vasculitis flare (19%). One-, 2-, 5- and 10-year overall survival rates were 91%, 89%, 79% and 65%, respectively. A prognostic score, the CryoVas score (CVS), for the prediction of survival at 5 years was devised. Pulmonary and gastrointestinal involvement, glomerular filtration rate 65 years were independently associated with death. At 5 years the death rates were 2.6%, 13.1%, 29.6% and 38.5% for a CVS of 0, 1, 2 and ≥3, respectively. At 1 year the death rates were 0%, 3.2%, 18.5% and 30.8% for a CVS of 0, 1, 2 and ≥3, respectively. The CVS was strongly correlated with the Five Factor Score (FFS) 2009, another prognostic score validated in primary necrotising vasculitis (r=0.82; p65 years, pulmonary and gastrointestinal involvement and renal failure. A score including these variables is significantly associated with the prognosis.
Published: 2013

43. Cardiac device implantation in Fabry disease

Author: Thierry Zenone, Joel Sebbah, Olivier Lidove, Olivier Fain, Jean-Marc Ziza, Thomas Sené, Laurent Aaron, Hervé Picard, Dominique Joly, Jean-Marc Darondel, and Philippe Charron
Subjects: medicine.medical_specialty, business.industry, Hypertrophic cardiomyopathy, Retrospective cohort study, General Medicine, Enzyme replacement therapy, 030204 cardiovascular system & hematology, medicine.disease, Ventricular tachycardia, Fabry disease, Sudden death, Surgery, Sudden cardiac death, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, Cardiology, 030212 general & internal medicine, business, Kidney disease
Abstract: The incidence and predictive factors of arrhythmias and/or conduction abnormalities (ACAs) requiring cardiac device (CD) implantation are poorly characterized in Fabry disease (FD). The aim of our retrospective study was to determine the prevalence, incidence, and factors associated with ACA requiring CD implantation in a monocentric cohort of patients with confirmed FD who were followed up in a department of internal medicine and reference center for FD.Forty-nine patients (20M, 29F) were included. Nine patients (4M, 5F; 18%) had at least one episode of ACA leading to device therapy. Six patients (4M/2F) required a pacemaker (PM) for sinus node dysfunction (n = 4) or atrioventricular disease (n = 2). One female patient required an internal cardioverter-defibrillator (ICD) to prevent sudden cardiac death because of nonsustained ventricular tachycardia (nSVT). One female patient required PM-ICD for sinus node dysfunction and nSVT. One patient underwent CD implantation before the diagnosis of FD. The annual rate of CD implantation was estimated at 1.90 per 100 person years. On univariate analysis at the end of the follow-up period, the factors associated with ACAs requiring CD implantation were as follows: delayed diagnosis of FD, delayed initiation of enzyme replacement therapy, age at the last follow-up visit, and severe multiorgan phenotype (hypertrophic cardiomyopathy, chronic kidney disease, and/or sensorineural hearing loss). On multivariate analysis, age at diagnosis of FD and age at the last follow-up visit were independently associated with an increased risk of ACAs requiring CD (P < 0.05).Considering the high frequency of ACAs requiring CD implantation and the risk of sudden death in patients with FD, regular monitoring is mandatory, especially in patients with a late diagnosis of FD and/or with a severe phenotype. Regular Holter ECGs, therapeutic education of patients, and deliverance of an emergency card including a phenotype summary are crucial in the care of FD patients.Available guidelines for device therapy and the efficacy of enzyme replacement therapy for arrhythmias or conduction abnormalities are discussed.
Published: 2016

44. [Cogan syndrome]

Author: Thierry, Zenone
Subjects: Decision Trees, Cogan Syndrome, Humans
Abstract: Cogan's syndrome is a rare disorder of unknown origin characterized by inflammatory ocular disease (mainly interstitial keratitis) and audiovestibular symptoms (mainly acute-onset sensorineural hearing loss) in the setting of a negative work-up for syphilis. The diversity of the ocular and audiovestibular manifestations reported in the literature in atypical Cogan's syndrome should make one cautious before accepting the diagnosis as the disease may mimic various other systemic disorders. Systemic corticosteroids are always the most widely used and successful therapy. For patients requiring high and prolonged doses, additional immunosuppression is appropriate. Methotrexate is the first-line steroid sparing agent. However, patients without systemic disease or severe eye disease unmanageable by topical corticosteroids should not be subjected to protracted courses of corticosteroids or immunosuppressive agents, particularly when little gain in hearing is obtained with their use. The effect of TNF-alpha blockers was recently investigated. Infliximab might be an alternative therapy in cases of failure of corticosteroids and immunosuppressive therapy. However, treatment might be more effective when started at an early stage of the disease, when the lesions are still reversible.
Published: 2012

45. Management of noninfectious mixed cryoglobulinemia vasculitis: data from 242 cases included in the CryoVas survey

Author: Antoine Huart, Estibaliz Lazaro, P. Belenotti, Olivier Hermine, David Launay, Guillaume Le Guenno, Thierry Zenone, Adeline Lacraz, Patrice Cacoub, Evguenia Krastinova, Franck Bridoux, Patricia Senet, P. Rullier, Elisabeth Diot, Xavier Mariette, Luc de Saint-Martin, Thomas Quemeneur, Isabelle Marie, Emmanuelle Plaisier, Benjamin Terrier, Fabrice Carrat, Fabrice Bonnet, Olivier Hinschberger, Jean-Emmanuel Kahn, Jean-Marc Léger, Immunologie - Immunopathologie - Immunothérapeutique (I3), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre de recherche en épidémiologie et santé des populations (CESP), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Médecine Interne [Rouen], Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Normandie Université (NU), Physiopathologie et biothérapies des maladies inflammatoires et autoimmunes, Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Department of Internal Medicine, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de Néphrologie-Transplantation-Dialyse, CHU Bordeaux [Bordeaux]-Groupe hospitalier Pellegrin, Service de Médecine Interne (MARSEILLE - Med Int), Assistance Publique - Hôpitaux de Marseille (APHM), Département de Médecine Interne et Pneumologie [Brest] (DMIP - Brest), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Groupe d'Etude de la Thrombose de Bretagne Occidentale (GETBO), Université de Brest (UBO)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO), Département de Médecine Interne (VALENCIENNES - Med Int), CH Valenciennes, Service de Néphrologie et Immunopathologie Clinique, Centre Hospitalier Universitaire de Toulouse, PRES Université de Toulouse, Foie, métabolismes et cancer, Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Centre Hospitalier Universitaire Estaing, CHU Estaing, Laboratoire d'Immunologie (EA 2686), Université de Lille, Droit et Santé, Service de Médecine Interne B, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Service de Médecine Interne, Hôpital Bretonneau, Service de médecine interne et maladies tropicales, CHU Bordeaux [Bordeaux]-Groupe hospitalier Saint-André, Contrôle de la Réponse Immune B et des Lymphoproliférations (CRIBL), Centre National de la Recherche Scientifique (CNRS)-Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503), Service de néphrologie - hémodialyse et transplantation rénale, Centre hospitalier universitaire de Poitiers (CHU Poitiers), Médecine Interne (VALENCE - Med Int), Centre hospitalier de Valence, Epidémiologie des maladies infectieuses et modélisation (ESIM), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Cytokines, hématopoïèse et réponse immune (CHRI), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de neurologie 1 [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Pitié-Salpêtrière [APHP], Régulation de la réponse immune, infection VIH-1 et autoimmunité, Université Paris-Sud - Paris 11 (UP11)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Dermatologie [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Tenon [APHP], Remodelage et Reparation du Tissu Renal, Service de médecine interne [CHU Pitié-Salpétrière], Biologie et thérapeutique des pathologies immunitaires (BTPI), Centre National de la Recherche Scientifique (CNRS)-Université Pierre et Marie Curie - Paris 6 (UPMC), Immunologie - Immunopathologie - Immunothérapeutique ( I3 ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Centre de recherche en épidémiologie et santé des populations ( CESP ), Université de Versailles Saint-Quentin-en-Yvelines ( UVSQ ) -Université Paris-Sud - Paris 11 ( UP11 ) -Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale ( INSERM ), CHU Rouen-Université de Rouen Normandie ( UNIROUEN ), Normandie Université ( NU ) -Normandie Université ( NU ), Université de Rouen Normandie ( UNIROUEN ), Normandie Université ( NU ) -Normandie Université ( NU ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Service de Médecine Interne ( MARSEILLE - Med Int ), Assistance Publique - Hôpitaux de Marseille ( APHM ), Département de Médecine Interne et Pneumologie [Brest] ( DMIP - Brest ), Centre Hospitalier Régional Universitaire de Brest ( CHRU Brest ), Groupe d'Etude de la Thrombose de Bretagne Occidentale ( GETBO ), Université de Brest ( UBO ), Département de Médecine Interne ( VALENCIENNES - Med Int ), Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Laboratoire d'Immunologie ( EA 2686 ), Centre Hospitalier Régional Universitaire [Montpellier] ( CHRU Montpellier ), Contrôle de la Réponse Immune B et des Lymphoproliférations ( CRIBL ), Centre National de la Recherche Scientifique ( CNRS ) -Université de Limoges ( UNILIM ) -Génomique, Environnement, Immunité, Santé, Thérapeutique ( GEIST FR CNRS 3503 ), Université de Poitiers-Centre hospitalier universitaire de Poitiers ( CHU Poitiers ), Médecine Interne ( VALENCE - Med Int ), Epidémiologie des maladies infectieuses et modélisation ( ESIM ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Cytokines, hématopoïèse et réponse immune ( CHRI ), Université Paris Descartes - Paris 5 ( UPD5 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Pitié-Salpêtrière [APHP], Université Paris-Sud - Paris 11 ( UP11 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Tenon [APHP], Biologie et thérapeutique des pathologies immunitaires ( BTPI ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Centre National de la Recherche Scientifique ( CNRS ), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris-Sud - Paris 11 (UP11)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Versailles Saint-Quentin-en-Yvelines (UVSQ), Service de Médecine Interne [CHU Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Université de Brest (UBO)-Université de Brest (UBO), CHU Clermont-Ferrand, Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503)-Centre National de la Recherche Scientifique (CNRS), Service de Neurologie [CHU Pitié-Salpêtrière], IFR70-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de dermatologie et allergologie [CHU Tenon], CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Université Pierre et Marie Curie - Paris 6 (UPMC)-Centre National de la Recherche Scientifique (CNRS), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Brestois Santé Agro Matière (IBSAM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service Médecine interne et immunopathologie clinique [CHU Toulouse], Pôle IUCT [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Université de Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Institut Brestois Santé Agro Matière (IBSAM), and Université de Brest (UBO)-Université de Brest (UBO)-Université de Brest (UBO)
Subjects: Male, MESH: Combined Modality Therapy, MESH : Retrospective Studies, [SDV]Life Sciences [q-bio], MESH : Aged, Arthritis, 030204 cardiovascular system & hematology, Biochemistry, Gastroenterology, MESH: Antibodies, Monoclonal, Cohort Studies, Antibodies, Monoclonal, Murine-Derived, 0302 clinical medicine, Prednisone, Adrenal Cortex Hormones, MESH : Female, MESH: Data Collection, MESH: Cohort Studies, MESH : Algorithms, MESH: Treatment Outcome, MESH: Aged, MESH: Middle Aged, Data Collection, MESH: Vasculitis, Antibodies, Monoclonal, Hematology, Middle Aged, Connective tissue disease, Combined Modality Therapy, 3. Good health, MESH : Vasculitis, MESH : Infection, Treatment Outcome, Cryoglobulinemia, MESH : Antibodies, Monoclonal, Rituximab, Female, medicine.symptom, MESH : Data Collection, Vasculitis, MESH: Infection, Algorithms, medicine.drug, medicine.medical_specialty, MESH: Cryoglobulinemia, MESH : Male, Immunology, MESH : Cohort Studies, MESH: Algorithms, MESH : Treatment Outcome, Infections, MESH : Adrenal Cortex Hormones, MESH: Adrenal Cortex Hormones, 03 medical and health sciences, Internal medicine, medicine, Humans, MESH : Middle Aged, Aged, Retrospective Studies, 030203 arthritis & rheumatology, MESH: Humans, [ SDV ] Life Sciences [q-bio], business.industry, MESH : Humans, MESH: Retrospective Studies, Cell Biology, Skin ulcer, medicine.disease, MESH : Cryoglobulinemia, MESH: Male, MESH : Antibodies, Monoclonal, Murine-Derived, Regimen, Peripheral neuropathy, MESH: Antibodies, Monoclonal, Murine-Derived, business, MESH : Combined Modality Therapy, MESH: Female
Abstract: Data on the clinical spectrum and therapeutic management of noninfectious mixed cryoglobulinemia vasculitis (CryoVas) in the era of hepatitis C virus screening are lacking. We analyzed data from 242 patients with noninfectious mixed CryoVas included in the French multicenter CryoVas survey. Baseline manifestations were purpura (75%), peripheral neuropathy (52%), arthralgia or arthritis (44%), glomerulonephritis (35%), cutaneous ulcers (16%), and cutaneous necrosis (14%). A connective tissue disease was diagnosed in 30% and B-cell non-Hodgkin lymphoma in 22%, whereas the CryoVas was considered to be essential in 48%. With the use of Cox-marginal structural models, rituximab plus corticosteroids showed the greater therapeutic efficacy compared with corticosteroids alone and alkylating agents plus corticosteroids to achieve complete clinical, renal, and immunologic responses and a prednisone dosage < 10 mg/d at 6 months. However, this regimen was also associated with severe infections, particularly when high doses of corticosteroids were used, whereas death rates did not differ between the therapeutic regimens. The role of each of these strategies remains to be defined in well-designed randomized controlled trials.
Published: 2012

46. Calcinosis and antisynthetase syndrome

Author: Thierry Zenone, Marie Puget, and Christelle Gibert
Subjects: medicine.medical_specialty, Myositis, business.industry, Electromyography, Hand Joints, Shoulder Joint, Calcinosis, Antisynthetase syndrome, Joint bone, Comorbidity, Middle Aged, medicine.disease, Radiography, Methotrexate, Treatment Outcome, Rheumatology, Azathioprine, medicine, Humans, Prednisone, Drug Therapy, Combination, Female, Radiology, business
Abstract: Joint Bone Spine - In Press.Proof corrected by the author Available online since mercredi 25 juillet 2012
Published: 2012

47. Dry cough is a frequent manifestation of giant cell arteritis

Author: Thierry Zenone and Marie Puget
Subjects: Male, medicine.medical_specialty, Pathology, Immunology, Giant Cell Arteritis, Rheumatology, Internal medicine, medicine, Immunology and Allergy, Humans, In patient, Elderly patient, Aged, Aged, 80 and over, business.industry, Dry cough, Acute-phase protein, Middle Aged, medicine.disease, Inflammatory biomarkers, Dermatology, respiratory tract diseases, Giant cell arteritis, Cough, Female, Vasculitis, business
Abstract: Physicians need to be familiar with the typical manifestations of giant cell arteritis. However, the challenge lies in recognizing atypical cases that lack the more specific manifestations or reflect vasculitis in less frequently involved territories. Among atypical clinical manifestations, dry cough has been reported in recent years. The literature contains sporadic reports mainly single case report. The objective of this study was to determine the frequency of dry cough in patients with giant cell arteritis. Clinical data were collected from 88 patients with giant cell arteritis. Relationships between dry cough and other clinical manifestations or biological data were analyzed. Dry cough of recent appearance was found at initial presentation of giant cell arteritis in 12 patients (13.6%). In 2 cases, dry cough was isolated. The 2 patients sought attention because of chronic dry cough associated with inflammation of unknown origin. In 10 cases, dry cough was associated with typical clinical manifestations of giant cell arteritis. A correlation was found between inflammatory biomarkers and presence of dry cough. The mean CRP was 153.8 mg/l (SD 85.1) in patients with dry cough and 94 mg/l (SD 72.2) in patients without dry cough (p = 0.0131). We conclude that the diagnosis of giant cell arteritis should always be considered in an elderly patient with an unexplained elevation of inflammatory markers and chronic dry cough. Dry cough in giant cell arteritis was not correlated with other clinical manifestations of this vasculitis, including pulmonary manifestations, but was correlated with inflammatory biomarkers.
Published: 2011

48. Preliminary criteria for the very early diagnosis of systemic sclerosis: results of a Delphi Consensus Study from EULAR Scleroderma Trials and Research Group

Author: Chris T. Derk, Wanda Maglione, Ileana Nicoara, Ulrich A. Walker, Alexandra Balbir-Gurman, Evelien Ton, R. E. de Souza, Branimir Anić, Roberto Caporali, L. Lonzetti, Jiri Stork, Daniela Opris, Ina Kötter, Stefan Heitmann, Stefano Bombardieri, J.M. van Laar, Evgeny Nasonov, Paul Hasler, Srdan Novak, S. Alhasani, Yannick Allanore, James R. Seibold, Murat Inanc, C. A. Von Mühlen, Raffaella Scorza, P. Eilbacher, G. Udrea, Oliver Distler, Brigitte Krummel-Lorenz, Britta Maurer, Otylia Kowal-Bielecka, Valeria Riccieri, Gianluca Moroncini, Alberto Sulli, Daniel E. Furst, Susanne Ullman, Y. Braun, Alessandro Mathieu, F. Stoeckl, I. Miniati, Percival D. Sampaio-Barros, Nemanja Damjanov, Henrik Nielsen, Patricia Carreira, Raffaele Pellerito, M. Buslau, Marco Matucci-Cerinic, E. De Langhe, Thierry Zenone, Peter Nash, D. Comina, Armando Gabrielli, Luc Mouthon, Jae Bum Jun, G. Riemekasten, Blaž Rozman, N. Del Papa, L. Alhajjar, Jutta G Richter, Jaap Fransen, Eric Hachulla, Stanislaw Sierakowsky, Miroslav Mayer, Małgorzata Widuchowska, Nicolas Hunzelmann, Ingo H. Tarner, M. Saracco, Coziana Ciurtin, Carlo Chizzolini, Maurizio Cutolo, P. Rehberger, Matthias Seidel, R. Ionitescu, Simona Rednic, Ulf Müller-Ladner, Paola Caramaschi, F.H.J. van den Hoogen, J.A. Pereira da Silva, Camillo Ribi, Christopher P. Denton, S. Bellando Randone, Magdalena Szmyrka-Kaczmarek, A Tyndall, Carmen Pizzorni, D. Launay, Jérôme Avouac, Rene Westhovens, Margitta Worm, Frank A. Wollheim, M. Lemos Lopes, C. Mihai, A. Sipek-Dolnicar, Alessandra Vacca, M. Meurer, Laura Bazzichi, Cord Sunderkötter, Cecília Varjú, Eugeniusz J. Kucharz, Søren Jacobsen, Vanessa Smith, Richard M. Silver, Gabriele Valentini, AT Kotulska, F De Keyser, M. Baresic, E. Rath, Marie Vanthuyne, Carolina de Souza Müller, S. Popa, Guido Valesini, Madelon C. Vonk, Dominique Farge, Ruxandra Ionescu, P. Coelho, B. Granel, A. Della Rossa, Maria João Salvador, T. Tourinho, Annegret Kuhn, Ewa Morgiel, C. Durant, L. Czirják, Maria Uprus, Tatiana Nevskaya, Paolo Amerio, Paolo Airò, Hans P. Kiener, D. Vealex, Avouac, J, Fransen, J, Walker, Ua, Riccieri, V, Smith, V, Muller, C, Miniati, I, Tarner, Ih, Randone, Sb, Cutolo, M, Allanore, Y, Distler, O, Valentini, Gabriele, Czirjak, L, MÜLLER LADNER, U, Furst, De, Tyndall, A, MATUCCI CERINIC, M, Eustar, Group, and University of Zurich
Subjects: medicine.medical_specialty, Delphi Technique, 2745 Rheumatology, Immunology, MEDLINE, Delphi method, 610 Medicine & health, Skin Diseases, General Biochemistry, Genetics and Molecular Biology, Scleroderma, Microscopic Angioscopy, Diagnosis, Differential, Fingers, Rheumatology, 1300 General Biochemistry, Genetics and Molecular Biology, medicine, Immunology and Allergy, Edema, Humans, skin and connective tissue diseases, computer.programming_language, Core set, 2403 Immunology, Scleroderma, Systemic, business.industry, 10051 Rheumatology Clinic and Institute of Physical Medicine, Raynaud Disease, medicine.disease, Surgery, Early Diagnosis, Family medicine, Antibodies, Antinuclear, Cohort, 2723 Immunology and Allergy, diagnostic criteria, early diagnosis, systemic sclerosis, Evaluation of complex medical interventions Auto-immunity, transplantation and immunotherapy [NCEBP 2], Observational study, business, computer, Delphi, Rheumatism
Abstract: ObjectiveTo identify a core set of preliminary items considered as important for the very early diagnosis of systemic sclerosis (SSc).MethodsA list of items provided by European League Against Rheumatism (EULAR) Scleroderma Trial and Research(EUSTAR) centres were subjected to a Delphi exercise among 110 experts in the field of SSc. In round 1, experts were asked to choose the items they considered as the most important for the very early diagnosis of SSc. In round 2, experts were asked to reconsider the items accepted after the first stage. In round 3, the clinical relevance of selected items and their importance as measures that would lead to an early referral process were rated using appropriateness scores.ResultsPhysicians from 85 EUSTAR centres participated in the study and provided an initial list of 121 items. After three Delphi rounds, the steering committee, with input from external experts, collapsed the 121 items into three domains containing seven items, developed as follows: skin domain (puffy fingers/puffy swollen digits turning into sclerodactily); vascular domain (Raynaud's phenomenon, abnormal capillaroscopy with scleroderma pattern) and laboratory domain (antinuclear, anticentromere and antitopoisomerase-I antibodies). Finally, the whole assembly of EUSTAR centres ratified with a majority vote the results in a final face-to-face meeting.ConclusionThe three Delphi rounds allowed us to identify the items considered by experts as necessary for the very early diagnosis of SSc. The validation of these items to establish diagnostic criteria is currently ongoing in a prospective observational cohort.
Published: 2010

49. Parotid gland non-Hodgkin lymphoma in primary Sjögren syndrome

Author: Thierry Zenone
Subjects: medicine.medical_specialty, Pathology, Biopsy, Immunology, Fatal Outcome, stomatognathic system, Rheumatology, immune system diseases, Predictive Value of Tests, hemic and lymphatic diseases, Internal medicine, medicine, Immunology and Allergy, Humans, Primary Sjögren Syndrome, B cell, Aged, medicine.diagnostic_test, business.industry, Lymphoma, B-Cell, Marginal Zone, Middle Aged, medicine.disease, Dermatology, Lymphoma, Parotid gland, Parotid Neoplasms, stomatognathic diseases, medicine.anatomical_structure, Sjogren's Syndrome, Treatment Outcome, Predictive value of tests, Female, Parotid Gland Non-Hodgkin Lymphoma, Lymphoma, Large B-Cell, Diffuse, Neoplasm Recurrence, Local, business, Tomography, X-Ray Computed, Immunosuppressive Agents
Abstract: The risk of malignant non-Hodgkin lymphoma is increased in primary Sjogren syndrome. In the literature, most studies evaluating this risk were conducted in tertiary reference university hospital. So, selection bias in series exists, in particular selection of the most severe cases in tertiary reference university care centers. Some studies had also a selection bias because patients were hospitalized (data were obtained from hospital discharge registries) and therefore the more severe cases were considered. Between October 1999 and November 2009, 109 adult patients were admitted to our department of internal medicine (non-university hospital, secondary level of the healthcare system, hospitalized patients and outpatient) with diagnosis of primary Sjogren syndrome. Two cases of parotid gland lymphoma occurred during the period of follow-up. No other lymphoma was detected. In this study, clinically identifiable parotid gland non-Hodgkin lymphoma occurs in 1.8% of patients with primary Sjogren syndrome. Because most non-Hodgkin lymphoma initially involves the neck organs, meticulous imaging studies mainly focused on the cervical regions are recommended in the follow-up of patients with primary Sjogren syndrome. Patients whose main complaint is persistent parotid gland swelling may have a parotid biopsy in order to diagnose non-Hodgkin lymphoma.
Published: 2010

50. Young woman with recurrent ischemic strokes diagnosed as Fabry disease: lessons learned from a case report

Author: Thierry Zenone and Victor Chan
Subjects: Adult, medicine.medical_specialty, Pediatrics, medicine.medical_treatment, Renal function, Kidney Function Tests, Brain Ischemia, Recurrent stroke, Recurrence, medicine, Humans, Enzyme Replacement Therapy, Thrombolytic Therapy, Intensive care medicine, Stroke, Proteinuria, business.industry, Ischemic strokes, General Medicine, Thrombolysis, medicine.disease, Fabry disease, Isoenzymes, Paresis, alpha-Galactosidase, Fabry Disease, Surgery, Female, Neurology (clinical), medicine.symptom, business, Tomography, X-Ray Computed, Kidney disease
Abstract: Several epidemiologic studies suggest that Fabry disease should be considered in young patients with cryptogenic stroke. We report a case of a young woman presenting with recurrent ischemic strokes who was finally diagnosed with Fabry disease after impaired kidney function had been identified. Fabry disease should be considered in unexplained cases of first or recurrent strokes in young patients disregarding the gender of the patient, especially when chronic kidney disease and/or proteinuria are present. Renal function should be closely monitored in patients with strokes and followed up after the event. In this case, intravenous thrombolysis was performed after the second ischemic event. No other case of thrombolysis for ischemic stroke in Fabry disease has been described in the literature.
Published: 2010

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