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116 results on '"Bernard Bonnotte"'

1. Splenectomy for primary immune thrombocytopenia revisited in the era of thrombopoietin receptor agonists: New insights for an old treatment

Author

Thibault Comont, Amandine Dernoncourt, Antoinette Perlat, Stéphane Cheze, Bernard Bonnotte, O. Souchaud-Debouverie, Pierre-Yves Jeandel, Bertrand Godeau, Jean-François Viallard, Jean-Christophe Lega, Delphine Gobert, Marc Michel, Mikael Ebbo, Corentin Orvain, Julie Graveleau, Antoine Dossier, Nathalie Costedoat-Chalumeau, Marc Ruivard, Louis Terriou, Arthur Mageau, CHU Henri Mondor, AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de référence des cytopénies auto-immunes [CHU Mondor] (CeReCAI), Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Université de Lille, CHU Lille, Hôpital de la Timone [CHU - APHM] (TIMONE), Aix Marseille Université (AMU), Centre hospitalier universitaire de Poitiers (CHU Poitiers), Innate Immunity and Immunotherapy (CRCINA-ÉQUIPE 7), Centre de Recherche en Cancérologie et Immunologie Nantes-Angers (CRCINA), Université d'Angers (UA)-Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Centre hospitalier universitaire de Nantes (CHU Nantes)-Université d'Angers (UA)-Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Centre hospitalier universitaire de Nantes (CHU Nantes), Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Fédérations hospitalo-universitaires Grand Ouest Acute Leukemia [Angers] (FHU GOAL), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Centre hospitalier de Saint-Nazaire, Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL), CHU Estaing [Clermont-Ferrand], CHU Clermont-Ferrand, Hôpital Haut-Lévêque [CHU Bordeaux], CHU Bordeaux [Bordeaux], Institut d'Hématologie de Basse-Normandie (IHBN), 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)-Centre Régional de Lutte contre le Cancer François Baclesse [Caen] (UNICANCER/CRLC), Normandie Université (NU)-UNICANCER-Tumorothèque de Caen Basse-Normandie (TCBN)-UNICANCER, CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), CHU Dijon, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Université de Bourgogne (UB), Hôpital Sud [CHU Rennes], CHU Pontchaillou [Rennes], CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Hôpital Cochin [AP-HP], Hôpital l'Archet, Université Côte d'Azur (UCA), Centre Hospitalier Universitaire de Nice (CHU Nice), CHU Amiens-Picardie, Institut Universitaire du Cancer de Toulouse - Oncopole (IUCT Oncopole - UMR 1037), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Bernardo, Elizabeth, CHU Henri Mondor [Créteil], Université de Toulouse (UT)-Université de Toulouse (UT)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Institut National de la Santé et de la Recherche Médicale (INSERM), and CHU Toulouse [Toulouse]

Subjects
Adult, Male, Thrombopoietin Receptor Agonists, medicine.medical_specialty, medicine.medical_treatment, Splenectomy, [SDV.CAN]Life Sciences [q-bio]/Cancer, Gastroenterology, [SDV.CAN] Life Sciences [q-bio]/Cancer, Internal medicine, Humans, Medicine, Retrospective Studies, Purpura, Thrombocytopenic, Idiopathic, business.industry, Retrospective cohort study, Hematology, Middle Aged, Immune thrombocytopenia, Treatment Outcome, Curative treatment, Sustained response, Female, Rituximab, business, Receptors, Thrombopoietin, medicine.drug
Abstract

Although splenectomy is still considered the most effective curative treatment for immune thrombocytopenia (ITP), its use has significantly declined in the last decade, especially since the approval of thrombopoietin receptor agonists (TPO-RAs). The main objective of the study was to determine whether splenectomy was still as effective nowadays, particularly for patients with failure to respond to TPO-RAs. Our secondary objective was to assess, among patients who relapsed after splenectomy, the pattern of response to treatments used before splenectomy. This multicentre retrospective study involved adults who underwent splenectomy for ITP in France from 2011 to 2020. Response status was defined according to international criteria. We included 185 patients, 100 (54.1%) and 135 (73.0%) patients had received TPO-RAs and/or rituximab before the splenectomy. The median follow-up after splenectomy was 39.2 months [16.5-63.0]. Overall, 144 (77.8%) patients had an initial response and 23 (12.4%) experienced relapse during follow-up, for an overall sustained response of 65.4%, similar to that observed in the pre-TPO-RA era. Among patients who received at least one TPO-RA or rituximab before splenectomy, 92/151 (60.9%) had a sustained response. Six of 13 (46%) patients with previous lack of response to both TPO-RAs and rituximab had a sustained response to splenectomy. Among patients with relapse after splenectomy, 13/21 (61.2%) patients responded to one TPO-Ras that failed before splenectomy. In conclusion, splenectomy is still a relevant option for treating adult primary ITP not responding to TPO-RAs and rituximab. Patients with lack of response or with relapse after splenectomy should be re-challenged with TPO-RAs. This article is protected by copyright. All rights reserved.

Published
2021

2. Myocardial infarction during giant cell arteritis: A cohort study

Author

Tibor Ponnelle, Sylvain Audia, Bernard Bonnotte, G. Muller, Alain Putot, Georges Tarris, André Ramon, Maxime Samson, Yves Cottin, Eric Steinmetz, Marianne Zeller, Nicolas Falvo, Béatrice Terriat, Maud Maza, Laurent Martin, Hélène Greigert, Catherine Creuzot-Garcher, Louis Arnould, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Interactions hôte-greffon-tumeur, ingénierie cellulaire et génique - UFC (UMR INSERM 1098) (RIGHT), 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]), Physiopathologie et épidémiologie cérébro-cardiovasculaire [Dijon] (PEC2), Université de Bourgogne (UB)-Université Bourgogne Franche-Comté [COMUE] (UBFC), Cypath [Dijon], Cypath : siège social [Villeurbanne], and The University Hospital of Dijon, the Association de Cardiologie de Bourgogne, and by grants from the Agence Regionale de Sante (ARS) de Bourgogne Franche-Comte, and from the Regional Council of Bourgogne Franche-Comte.

Subjects
medicine.medical_specialty, Population, Coronary Artery Disease, 030204 cardiovascular system & hematology, Cohort Studies, Coronary artery disease, 03 medical and health sciences, 0302 clinical medicine, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, immune system diseases, Prednisone, Internal medicine, Internal Medicine, medicine, Humans, cardiovascular diseases, 030212 general & internal medicine, Myocardial infarction, skin and connective tissue diseases, education, education.field_of_study, population study, giant cell arteritis, business.industry, medicine.disease, 3. Good health, Giant cell arteritis, myocardial infarction, Cohort, cardiovascular system, Cardiology, Population study, France, business, medicine.drug, Cohort study
Abstract

International audience; BACKGROUND: Cardiovascular risk is increased in giant cell arteritis (GCA). We aimed to characterize myocardial infarction (MI) in a GCA cohort, and to compare the GCA and non-GCA population affected by MI. METHODS: In patients with a biopsy-proven diagnosis of GCA between 1 January 2001 and 31 December 2016 in Côte D'Or (France), we identified patients with MI by crossing data from the territorial myocardial infarction registry (Observatoire des Infarctus de Côte d'Or) database. Five controls (non-GCA + MI) were paired with one case (GCA + MI) after matching for age, sex, cardiovascular risk factors and prior cardiovascular disease. MI were characterized as type 1 MI (T1MI), resulting from thrombus formation due to atherothrombotic disease, or type 2 MI (T2MI), due to a myocardial supply/demand mismatch. GCA-related MI was defined as MI occurring within 3 months of a GCA flare (before or after). RESULTS: Among 251 biopsy-proven GCA patients, 13 MI cases were identified and paired with 65 controls. MI was GCA-related in 6/13 cases, accounting for 2.4% (6/251) of our cohort. T2MI was more frequently GCA-related than GCA-unrelated (80% vs. 16.7%, p = 0.080), and GCA diagnosis was the only identified triggering factor in 75% of GCA-related T2MI. GCA-unrelated MI were more frequently T1MI and occurred in patients who had received a higher cumulative dose of prednisone (p = 0.032). GCA was not associated with poorer one-year survival. CONCLUSIONS: GCA-related MI are mainly T2MI probably caused by systemic inflammation rather than coronaritis. GCA-unrelated MI are predominantly T1MI associated with atherothrombotic coronary artery disease.

Published
2021

3. COVID-19 or not COVID-19? Compared characteristics of patients hospitalized for suspected COVID-19

Author

Pascal Chavanet, Marielle Buisson, Isabelle Eberl, Lucas Mathey, François Xavier Catherine, M. Blot, Thibault Sixt, Lionel Piroth, Barbara Nicolas, Thomas Rogier, S. Mahy, Leila Benguella, Clémentine Estève, Arnaud Salmon-Rousseau, Antoine Coussement, Bernard Bonnotte, Florian Moretto, Lucile Behague, Quentin Bernard, Michel Duong, and Maroua Abdallahoui

Subjects
Adult, Male, 0301 basic medicine, Microbiology (medical), myalgia, medicine.medical_specialty, Multivariate analysis, Patients, COVID-19, 030106 microbiology, Fibrinogen, SARS-CoV2, 03 medical and health sciences, 0302 clinical medicine, Medical microbiology, Internal medicine, Tobacco, medicine, Humans, 030212 general & internal medicine, Infectious disease (athletes), Medical diagnosis, Aged, Retrospective Studies, Differential diagnosis, SARS-CoV-2, business.industry, Brief Report, General Medicine, medicine.disease, Hospitalization, Infectious Diseases, Heart failure, medicine.symptom, business, medicine.drug
Abstract

During an epidemic period, we compared patients hospitalized for initial suspicion of COVID-19 but for whom an alternative diagnosis was finally retained (n = 152) with those who had COVID-19 (n = 222). Most common diagnoses were another infectious disease and heart failure. COVID-19-negative patients were more often active smokers had less often cough, fever, and digestive symptoms, as compared to the 222 COVID-19-positive patients. They had higher median neutrophil and lymphocyte counts and lower CRP level. In multivariate analysis, no current smoking, neurocognitive disorder, myalgia, and fibrinogen ≥4g/L were independently associated with a final diagnosis of COVID-19.

Published
2021

4. Traitements de première ligne au cours du purpura thrombopénique immunologique de l'adulte : état des lieux et perspectives

Author

Bertrand Godeau, Bernard Bonnotte, and Marc Michel

Subjects
medicine.medical_specialty, medicine.drug_class, business.industry, Gastroenterology, medicine.disease, Monoclonal antibody, Thrombocytopenic purpura, Immune system, Prednisone, Intravenous Immunoglobulins, hemic and lymphatic diseases, Internal medicine, Internal Medicine, medicine, Corticosteroid, Platelet, business, Dexamethasone, medicine.drug
Abstract

The first line treatment of immune thrombocytopenic purpura (ITP) is well established and based on short course of corticosteroids associated with intravenous immunoglobulins (IVIg) for the most severe forms. Predniso(lo)ne is the corticosteroid agent usually given but dexamethasone appears as an alternative. Some guidelines recommend to use dexamethasone as first line when a rapid increase of platelet count is required. Dexamethasone could be used rather than IVIg for moderate to severe but non life-threatening bleeding manifestations. Other therapeutic options such as anti FcRn monoclonal antibodies or recombinant FcγR currently in development for ITP could be an option in the future. In newly diagnosed ITP, we unfortunately lack robust predictive risk factors of severity and chronic outcome. Identifying such factors could be helpful for considering the early use of some treatments which are commonly used as second or third line.

Published
2021

5. Localized versus systemic granulomatosis with polyangiitis: data from the French Vasculitis Study Group Registry

Author

Claire de Moreuil, Olivier Aumaître, Pascal Godmer, Alexis Régent, François Maurier, Christian Pagnoux, Luc Mouthon, Bernard Bonnotte, Xavier Puéchal, Michele Iudici, Chahéra Khouatra, Noémie Jourde-Chiche, Marc Ruivard, François Lifermann, Benjamin Terrier, Claire Blanchard-Delaunay, Antoine Néel, Eric Hachulla, Pascal Cohen, Delphine S. Courvoisier, Thomas Le Gallou, Jean-François Viallard, Alain Le Quellec, Achille Aouba, Loïc Guillevin, Thomas Quemeneur, Alexandre Karras, Centre recherche en CardioVasculaire et Nutrition = Center for CardioVascular and Nutrition research (C2VN), and Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)

Subjects
Vasculitis, medicine.medical_specialty, Cyclophosphamide, Subglottic stenosis, [SDV]Life Sciences [q-bio], Context (language use), macromolecular substances, vasculitis, Antibodies, Antineutrophil Cytoplasmic, stomatognathic system, Rheumatology, Recurrence, Internal medicine, medicine, Humans, Pharmacology (medical), Registries, Sinusitis, Retrospective Studies, ddc:616, Lung, granulomatosis with polyangiitis, business.industry, medicine.disease, Otitis, medicine.anatomical_structure, Granulomatosis with polyangiitis, medicine.symptom, business, ANCA-associated vasculitis, medicine.drug
Abstract

Objective To describe the main features at diagnosis and evolution over time of patients with localized granulomatosis with polyangiitis (L-GPA) compared with those of systemic GPA (S-GPA). Methods EULAR definitions of L-GPA, i.e. upper and/or lower respiratory tract involvement, and S-GPA were applied to patients from the French Vasculitis Study Group Registry. L-GPA and S-GPA patients’ characteristics at diagnosis and long-term outcomes were analysed and compared. Results Among the 795 Registry patients, 79 (10%) had L-GPA. Their main clinical manifestations were rhinitis, lung nodules, sinusitis and otitis. L-GPA vs S-GPA patients at diagnosis, respectively, were younger, more frequently had saddle nose deformity or subglottic stenosis and were less often PR3-ANCA–positive. L-GPA vs S-GPA induction therapy less frequently included CYC but more often a combination of MTX and glucocorticoids; 64% of MTX-treated patients experienced disease progression within 18 months post-diagnosis. L- and S-GPA patients’ estimated relapse-free–survival probabilities, relapse rates and refractory disease rates at each time point were comparable, but L-GPA patients had more frequent ENT and lung relapses, and higher overall survival rates (P Conclusions The relapse risks of L-GPA and S-GPA were similar, but relapse patterns differed and L-GPA overall survival rate was higher. About one-quarter of L-GPA patients developed S-GPA over time, but without end-stage organ involvement.

Published
2022

6. Patterns of fatigue and association with disease activity and clinical manifestations in systemic lupus erythematosus

Author

Jean Sibilia, Reinhard E. Voll, Hanns-Martin Lorenz, Thierry Martin, Bernard Bonnotte, Gilles Blaison, Zahir Amoura, Anne-Sophie Korganow, Andreas Schwarting, Laurent Arnaud, Vincent Poindron, Philippe Mertz, François Maurier, and Christoph Fiehn

Subjects
Adult, Male, medicine.medical_specialty, Databases, Factual, Mixed anxiety-depressive disorder, Anxiety, Cohort Studies, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Interquartile range, Internal medicine, Prevalence, medicine, Cluster Analysis, Humans, Lupus Erythematosus, Systemic, Pharmacology (medical), 030212 general & internal medicine, Fatigue, Depression (differential diagnoses), 030203 arthritis & rheumatology, Systemic lupus erythematosus, Lupus erythematosus, Depression, business.industry, Middle Aged, medicine.disease, Latent class model, Patient Outcome Assessment, Cohort, Female, medicine.symptom, business
Abstract

Objective The prevalence of fatigue is high in patients with systemic lupus erythematosus (SLE). In this study, we used latent class analysis to reveal patterns of fatigue, anxiety, depression and organ involvement in a large international cohort of SLE patients. Methods We used the Lupus BioBank of the upper Rhein to analyse patterns of fatigue using latent class analysis (LCA). After determining the optimal number of latent classes, patients were assigned according to model generated probabilities, and characteristics of classes were compared. Results A total of 502 patients were included. Significant fatigue, anxiety and depression were reported by 341 (67.9%), 159 (31.7%) and 52 (10.4%) patients, respectively. LCA revealed a first cluster (67.5% of patients) with low disease activity [median (25th–75th percentile interquartile range) Safety of Estrogens in Lupus Erythematosus National Assessment (SELENA)-SLEDAI: 2 (0–4)], significant fatigue (55.5%, P Conclusion LCA revealed three patterns of fatigue with important practical implications. Based on these, it is crucial to distinguish patients with active disease (in whom remission will be achieved) from those with no or mild activity but high levels of fatigue, depression and anxiety, for whom psychological counselling should be prioritized.

Published
2020

7. Type 1 Diabetes in People Hospitalized for COVID-19: New Insights From the CORONADO Study

Author

Sandrine Lablanche, Florence Galtier, Pierre Leroy, Vincent Dubée, Dominique Bonnefont-Rousselot, Patrick Saulnier, Matthieu Wargny, Manuel Etienne, Patrice Darmon, Anne-Laure Borel, Jean-Louis LAPLANCHE, Olivier Lesieur, Bertrand Cariou, Malak Taher, Lydia GUITTET, Vincent Minville, BRUNO MOURVILLIER, Jean-Pierre QUENOT, Bernard Bonnotte, Lucien Marchand, Team2 Carmen, Veronique Kerlan, Pascal Reynier, Nadia Sabbah, Matthieu PICHELIN, Jean-Charles Aurégan, Guillaume Martin-Blondel, Olivier Bourron, Blandine Rammaert, Pierre-Edouard Fournier, Coralie Amadou, Giulia Chinetti, Jean-Christophe Lagier, Didier Raoult, Centre hospitalier universitaire de Nantes (CHU Nantes), Institut des Maladies Métaboliques et Cardiovasculaires (I2MC), Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT)-Université de Toulouse (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre de Recherche des Cordeliers (CRC (UMR_S_1138 / U1138)), École Pratique des Hautes Études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Université Paris Cité (UPCité), Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Research Unit on Cardiovascular and Metabolic Diseases (ICAN), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Institut de Cardiométabolisme et Nutrition = Institute of Cardiometabolism and Nutrition [CHU Pitié Salpêtrière] (IHU ICAN), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Pitié-Salpêtrière [AP-HP], Hôpital de la Conception [CHU - APHM] (LA CONCEPTION), Centre recherche en CardioVasculaire et Nutrition = Center for CardioVascular and Nutrition research (C2VN), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Centre Hospitalier Sud Francilien, Unité de recherche de l'institut du thorax (ITX-lab), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN), Service d'endocrinologie, diabétologie et nutrition [CHU Bichat], 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)-Université Paris Cité (UPCité), Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA), Cardiovasculaire, métabolisme, diabétologie et nutrition (CarMeN), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Hospices Civils de Lyon (HCL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Hôpital Lariboisière-Fernand-Widal [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Recherches Translationnelles sur le VIH et les maladies infectieuses endémiques et émergentes (TransVIHMI), Institut de Recherche pour le Développement (IRD)-Université de Yaoundé I-Université Cheikh Anta Diop [Dakar, Sénégal] (UCAD)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Pathogénèse et contrôle des infections chroniques (PCCI), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Diabétologie [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Université de Paris (UP), Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Institute of cardiometabolism and nutrition (ICAN), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), CH Evry-Corbeil, unité de recherche de l'institut du thorax UMR1087 UMR6291 (ITX), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université de Paris (UP), Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hospices Civils de Lyon (HCL), Recherches Translationnelles sur le VIH et les maladies infectieuses endémiques er émergentes (TransVIHMI), Université Cheikh Anta Diop [Dakar, Sénégal] (UCAD)-Institut de Recherche pour le Développement (IRD)-Université de Yaoundé I-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Université Montpellier 1 (UM1), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Centre Hospitalier Universitaire de Montpellier (CHU Montpellier ), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées, Service de diabétologie [CHU Pitié-Salpétrière], Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université de Paris (UP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université de Montpellier (UM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), and Gestionnaire, Hal Sorbonne Université

Subjects
Male, Pediatrics, medicine.medical_specialty, [SDV]Life Sciences [q-bio], Endocrinology, Diabetes and Metabolism, Pneumonia, Viral, Population, 030209 endocrinology & metabolism, law.invention, 03 medical and health sciences, 0302 clinical medicine, Risk Factors, law, Diabetes mellitus, Internal Medicine, medicine, Clinical endpoint, Humans, 030212 general & internal medicine, education, Pandemics, Case report form, ComputingMilieux_MISCELLANEOUS, Aged, Aged, 80 and over, Glycated Hemoglobin, Advanced and Specialized Nursing, Inpatients, Type 1 diabetes, education.field_of_study, business.industry, e-Letters: Observations, COVID-19, Middle Aged, Prognosis, medicine.disease, Respiration, Artificial, Intensive care unit, 3. Good health, [SDV] Life Sciences [q-bio], Diabetes Mellitus, Type 2, Hypertension, Etiology, Female, Observational study, Coronavirus Infections, business
Abstract

Since the start of the coronavirus disease 2019 (COVID-19) pandemic, patients with diabetes were rapidly recognized as a high-risk population for severe disease. Indeed, a high prevalence of diabetes among patients with COVID-19 who required hospitalization has been consistently reported, reaching 33.8% in 5,700 people hospitalized for COVID-19 in the New York City area (1). In addition, diabetes was associated with more than a doubled risk of intensive care unit (ICU) admission and more than a tripled risk of death (2). However, precise data regarding the type of diabetes are scarce. We report here the clinical characteristics and early prognosis of patients with type 1 diabetes (T1D) hospitalized for COVID-19 in the nationwide multicenter observational CORONADO (Coronavirus SARS-CoV-2 and Diabetes Outcomes) study (3). The aim of the CORONADO study was to describe the phenotypic characteristics and prognosis of patients with diabetes admitted with COVID-19 between 10 March and 10 April 2020 in 68 French hospitals. The protocol (ClinicalTrials.gov reg. no. NCT04324736) obtained all regulatory approvals as recently described (3). Inclusion criteria were 1 ) hospitalization for biologically and/or clinically/radiologically attested COVID-19 and 2 ) personal history of diabetes or newly diagnosed diabetes on admission. The composite primary end point combined tracheal intubation for mechanical ventilation and/or death on day 7 (D7). Secondary outcomes included death, tracheal intubation, and discharge on D7. Classification of diabetes was recorded in the electronic case report form as noted in the medical file by the physician in charge of the patient. In the present subanalysis, patients in whom diabetes was diagnosed on admission were excluded since the etiological diagnosis had not been formally established. All cases noted as T1D were carefully reviewed by local investigators and the steering committee …

Published
2020

8. Long-Term Rituximab Use to Maintain Remission of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

Author

Alexandre Karras, Stéphane Vinzio, Catherine Hanrotel-Saliou, Grégory Pugnet, Benjamin Terrier, Nadine Meaux-Ruault, Eric Hachulla, Antoine Huart, Elodie Perrodeau, Christian Agard, Bernard Bonnotte, Thomas Le Gallou, Philippe Ravaud, Maxime Samson, Nicolas Martin-Silva, Christine Vinter, Xavier Puéchal, Pierre Charles, Jean Sibilia, Antoine Néel, Jean-François Viallard, Loïc Guillevin, P. Gobert, François Maurier, François Lifermann, Luc Mouthon, Pascal Godmer, Pascal Cohen, and Pierre-Louis Carron

Subjects
medicine.medical_specialty, business.industry, 010102 general mathematics, General Medicine, medicine.disease, Placebo, 01 natural sciences, law.invention, 03 medical and health sciences, Regimen, 0302 clinical medicine, Randomized controlled trial, Maintenance therapy, law, Internal medicine, Internal Medicine, medicine, Rituximab, 030212 general & internal medicine, 0101 mathematics, Microscopic polyangiitis, business, Granulomatosis with polyangiitis, medicine.drug, Anti-neutrophil cytoplasmic antibody
Abstract

Background Biannual rituximab infusions over 18 months effectively maintain remission after a "standard" remission induction regimen for patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Objective To evaluate the efficacy of prolonged rituximab therapy in preventing AAV relapses in patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) who have achieved complete remission after completing an 18-month maintenance regimen. Design Randomized controlled trial. (ClinicalTrials.gov: NCT02433522). Setting 39 clinical centers in France. Patients 68 patients with GPA and 29 with MPA who achieved complete remission after the first phase of maintenance therapy. Intervention Rituximab or placebo infusion every 6 months for 18 months (4 infusions). Measurements The primary end point was relapse-free survival at month 28. Relapse was defined as new or reappearing symptoms or worsening disease, with a Birmingham Vasculitis Activity Score greater than 0. Results From March 2015 to April 2016, 97 patients (mean age, 63.9 years; 35% women) were randomly assigned, 50 to the rituximab and 47 to the placebo group. Relapse-free survival estimates at month 28 were 96% (95% CI, 91% to 100%) and 74% (CI, 63% to 88%) in the rituximab and placebo groups, respectively, an absolute difference of 22% (CI, 9% to 36%) with a hazard ratio of 7.5 (CI, 1.67 to 33.7) (P = 0.008). Major relapse-free survival estimates at month 28 were 100% (CI, 93% to 100%) versus 87% (CI, 78% to 97%) (P = 0.009), respectively. At least 1 serious adverse event developed in 12 patients (24%) in the rituximab group (with 9 infectious serious adverse events occurring among 6 patients [12%]) versus 14 patients (30%) in the placebo group (with 6 infectious serious adverse events developing among 4 patients [9%]). No deaths occurred in either group. Limitation Potential selection bias based on previous rituximab response and tolerance. Conclusion Extended therapy with biannual rituximab infusions over 18 months was associated with a lower incidence of AAV relapse compared with standard maintenance therapy. Primary funding source French Ministry of Health and Hoffmann-La Roche.

Published
2020

9. Reducing the initial number of rituximab maintenance-therapy infusions for ANCA-associated vasculitides: randomized-trial post-hoc analysis

Author

Olivier Aumaître, Xavier Puéchal, Grégory Pugnet, Pascal Godmer, François Maurier, P. Gobert, Stanislas Faguer, Mohamed Hamidou, François Lifermann, Sophie Rivière, Luc Mouthon, Noémie Jourde-Chiche, Bernard Bonnotte, Benjamin Terrier, Christian Agard, Nadine Meaux-Ruault, Loïc Guillevin, Agnès Dechartres, Antoine Huart, Nicolas Martin-Silva, Jean Sibilia, Alexandre Karras, Pierre Charles, Maxime Samson, Marie Matignon, Pascal Cohen, Jean-François Viallard, and Catherine Hanrotel-Saliou

Subjects
medicine.medical_specialty, Randomization, Antigens, CD19, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Gastroenterology, Disease-Free Survival, Drug Administration Schedule, Antibodies, Antineutrophil Cytoplasmic, Maintenance Chemotherapy, law.invention, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Maintenance therapy, Randomized controlled trial, law, Internal medicine, Post-hoc analysis, medicine, Humans, Pharmacology (medical), 030212 general & internal medicine, Survival rate, 030203 arthritis & rheumatology, business.industry, medicine.disease, Antirheumatic Agents, Rituximab, Microscopic polyangiitis, Granulomatosis with polyangiitis, business, medicine.drug
Abstract

ObjectiveThe randomized, controlled MAINRITSAN2 trial was designed to compare the capacity of an individually tailored therapy [randomization day 0 (D0)], with reinfusion only when CD19+ lymphocytes or ANCA had reappeared, or if the latter’s titre rose markedly, with that of five fixed-schedule 500-mg rituximab infusions [D0 + D14, then months (M) 6, 12 and 18] to maintain ANCA-associated vasculitis (AAV) remissions. Relapse rates did not differ at M28. This ancillary study was undertaken to evaluate the effect of omitting the D14 rituximab infusion on AAV relapse rates at M12.MethodsMAINRITSAN2 trial data were subjected to post-hoc analyses of M3, M6, M9 and M12 relapse-free survival rates in each arm as primary end points. Exploratory subgroup analyses were run according to CYC or rituximab induction and newly diagnosed or relapsing AAV.ResultsAt M3, M6, M9 and M12, respectively, among the 161 patients included, 79/80 (98.8%), 76/80 (95%), 74/80 (92.5%) and 73/80 (91.3%) from D0, and 80/81 (98.8%), 78/81 (96.3%), 76/81 (93.8%) and 76/81 (93.8%) from D0+D14 groups were alive and relapse-free. No between-group differences were observed. Results were not affected by CYC or rituximab induction, or newly diagnosed or relapsing AAV.ConclusionsWe were not able to detect a difference between the relapse-free survival rates for up to M12 for the D0 and D0+D14 rituximab-infusion groups, which could suggest that omitting the D14 rituximab remission-maintenance dose did not modify the short-term relapse-free rate. Nevertheless, results at M12 may also have been influenced by the rituximab-infusion strategies for both groups.

Published
2020

10. Case Report: Evolution of a Severe Vascular Refractory Form of ECD Requiring Liver Transplantation Correlated With the Change in the Monocyte Subset Analysis

Author

Francine Garnache-Ottou, Thibaud Soumagne, Bernard Bonnotte, Jerome Razanamahery, Anne Roggy, Julien Haroche, Jean-François Emile, Zaher Lakkis, Alexandre Malakhia, Fleur Cohen-Aubart, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Etablissement français du sang [Bourgogne-Franche-Comté] (EFS [Bourgogne-Franche-Comté]), Hôpital Ambroise Paré [AP-HP], Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Service de Médecine Interne 2, maladies auto-immunes et systémiques [CHU Pitié-Salpêtrière], Institut E3M [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)-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)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], and Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)

Subjects
Pathology, medicine.medical_specialty, Myeloid, CD14, Immunology, Erdheim–Chester disease, Case Report, CD16, Pathogenesis, 03 medical and health sciences, 0302 clinical medicine, medicine, Immunology and Allergy, 030304 developmental biology, 0303 health sciences, business.industry, Monocyte, RC581-607, medicine.disease, 3. Good health, Transplantation, Histiocytosis, Vascular diagnosis, medicine.anatomical_structure, 030220 oncology & carcinogenesis, monocyte, Erdheim-Chester disease, Immunologic diseases. Allergy, business, histiocytosis, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, transplantation
Abstract

Erdheim–Chester disease is a rare histiocytosis characterized by iconic features associated with compatible histology. Most patients have somatic mutations in the MAP-kinase pathway gene, and the mutations occur in CD14+ monocytes. Differentiation of the myeloid lineage plays a central role in the pathogenesis of histiocytosis. Monocytes are myeloid-derived white blood cells, divided into three subsets, but only the CD14++CD16− “classical monocyte” can differentiate into dendritic cells and tissue macrophages. Since most mutations occur in CD14+ cells and since ECD patients have a particular monocytic phenotype resembling CMML, we studied the correlation between disease activity and monocytic subset distribution during the course of a severe vascular form of ECD requiring liver transplantation. During early follow-up, increased CD14++CD16− “classical monocyte” associated with decreased CD14lowCD16++ “non-classical monocyte” correlated with disease activity. Further studies are needed to confirm the use of monocyte as a marker of disease activity in patients with ECD.

Published
2021

11. Eltrombopag in adult patients with immune thrombocytopenia in the real-world in France, including off-label use before 6 months of disease duration: The multicenter, prospective ELEXTRA study

Author

Julia Moeglin, Morgane Mourguet, Laurent Alric, Laure Swiader, Suzanne Tavitian, Jean-Marc Durand, Sylvie Ollier, Aline Moignet‐Autrel, Karen Delavigne, Miguel Carreiro, Pierre Cougoul, Jean Estelle, Sylvain Audia, Benjamin De Sainte Marie, Veronique Remy, Xavier Delbrel, Samuel Deshayes, Francis Gaches, Helene Hennique, Lorraine Leplay, Gaetan Sauvetre, Antoine Briantais, Cécile Borel, Kamel Laribi, Martin Michaud, Stéphane Cheze, Sondess Hadj‐Khelifa, Guillaume Martin‐Blondel, Geoffrey Urbanski, Brice Castel, Guillaume Moulis, Fanny Nuccio, Soraya Leclerc-Teffahi, Odile Beyne‐Rauzy, Benjamin Hebraud, Aurelie Godel‐Labouret, Pierre Duffau, Sarah Khatibi, Baptiste Andre, Gregory Pugnet, Daniel Adoue, Julie Seguier, Clement Gaudin, Marc Michel, Myriam Aroichane, Laurent Prudhomme, Margaux Lafaurie, Manuela Rueter, Philippe Montane De La Roque, Natacha Brun, Aurelie Saunier, Julie Graveleau, Johanne Germain, Willy Vaillant, Agnès Sommet, Maryse Lapeyre-Mestre, Delphine Bonnet, Stephane Sire, Yann Leveneur, Caroline Soubrier, Alina Danu, Veronique Veit, Patrick Giraud, François Lifermann, Gwenola Maigne, Jean-François Viallard, Clothilde Martel, Nicolas Limal, Delphine Brechemier, Frederique Roy‐Peaud, Serge Madaule, Laurent Sailler, Thibault Comont, Benoit Faucher, Laurent Balardy, Carine Courtault, Claire Dingremont, Jean-Robert Harlé, Sophie Arista, Mikael Ebbo, Bertrand Godeau, Jeremie Dion, Bernard Bonnotte, Irène Machelart, Matthieu Mahévas, Nicolas Schleinitz, Arnaud Saint‐Lezer, Corentin Orvain, Christian Recher, Patrick Rispal, and Bertrand Lioger

Subjects
Adult, Male, Pediatrics, medicine.medical_specialty, Disease duration, Eltrombopag, Off-label use, Benzoates, chemistry.chemical_compound, medicine, Humans, Prospective Studies, Aged, Purpura, Thrombocytopenic, Idiopathic, Adult patients, business.industry, Hematology, Off-Label Use, Middle Aged, Immune thrombocytopenia, Hydrazines, Treatment Outcome, chemistry, Pyrazoles, Female, France, business
Published
2021

12. Hematological malignancies in giant cell arteritis: a french population-based study

Author

Bernard Bonnotte, Georges Tarris, André Ramon, Maxime Samson, Morgane Mounier, Sylvain Audia, Marc Maynadié, Hélène Greigert, Louis Arnould, Catherine Creuzot-Garcher, Laurent Martin, Tibor Ponnelle, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), 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 [Bourgogne-Franche-Comté])-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC), Service de médecine interne et immunologie clinique (SOC 1) [CHU de Dijon], Laboratoire d'Excellence : Lipoprotéines et Santé : prévention et Traitement des maladies Inflammatoires et du Cancer (LabEx LipSTIC), École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Institut Gustave Roussy (IGR)-Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)-Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon)-Université de Bourgogne (UB)-Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon)-Centre Régional de Lutte contre le cancer Georges-François Leclerc [Dijon] (UNICANCER/CRLCC-CGFL), UNICANCER-UNICANCER-Institut National de la Santé et de la Recherche Médicale (INSERM)-Fédération Francophone de la Cancérologie Digestive, FFCD-Université de Montpellier (UM)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Etablissement français du sang [Bourgogne-Franche-Comté] (EFS [Bourgogne-Franche-Comté])-Centre National de la Recherche Scientifique (CNRS)-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Registre des hémopathies malignes de Côte d'Or, Lipides - Nutrition - Cancer [Dijon - U1231] (LNC), Université de Bourgogne (UB)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Institut National de la Santé et de la Recherche Médicale (INSERM), Service d'Ophtalmologie (CHU de Dijon), Cypath [Dijon], Cypath : siège social [Villeurbanne], Service d'hématologie biologique (CHU de Dijon), Julien, Sabine, and Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement

Subjects
Male, 0301 basic medicine, medicine.medical_specialty, Myeloid, Population, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, immune system diseases, Internal medicine, Epidemiology, medicine, Humans, Pharmacology (medical), cardiovascular diseases, hematological malignancies, [SDV.MHEP.OS]Life Sciences [q-bio]/Human health and pathology/Sensory Organs, education, skin and connective tissue diseases, Retrospective Studies, 030203 arthritis & rheumatology, education.field_of_study, business.industry, giant cell arteritis, Incidence, Incidence (epidemiology), Middle Aged, medicine.disease, 3. Good health, Population based study, Giant cell arteritis, 030104 developmental biology, medicine.anatomical_structure, Increased risk, [SDV.MHEP.OS] Life Sciences [q-bio]/Human health and pathology/Sensory Organs, population-based study, Hematologic Neoplasms, cardiovascular system, Female, France, business
Abstract

Objectives An increased risk of haematological malignancies (HM) has been reported in GCA patients. Our study aimed to investigate the incidence and the type of HM occurring in GCA. Methods All patients with GCA and HM living in Côte d’Or (France) were identified by crossing data from the RHEMCO (Registre des Hémopathies Malignes de Côte d’Or) and those having a positive temporal artery biopsy between 1 January 2001 and 31 December 2018. Results Among 276 biopsy-proven GCA patients, 14 HM were identified in 12 patients (4.3%). In comparison with the general population aged >50 y, the incidence of myeloid HM and myeloproliferative syndromes were increased in GCA patients [standardized incidence ratios (SIR) = 2.71 and 5.16, respectively], with a specific increase in men with GCA (SIR = 4.82 and 9.04, respectively) but not in women. In addition, the study of SIR depending on the chronology between GCA and HM diagnoses suggests that there was an increased risk of developing GCA in men but not in women, after a diagnosis of myeloid HM (SIR = 9.56), especially if it was a MPS (SIR = 17.56). Conclusions Our study shows a particular epidemiology of HM in GCA patients, which is characterized by an increased incidence of myeloid HM, especially MPS, in male GCA patients. The chronology of the diagnoses of GCA and HM raises the hypothesis that clonal hematopoiesis may be implicated in some cases of GCA.

Published
2021

13. Alpelisib administration reduced lymphatic malformations in a mouse model and in patients

Author

Gabriel Morin, Patrick Villarese, Clément Hoguin, Olivia Boccara, Quitterie Venot, Bernard Bonnotte, Vahid Asnafi, Tristan Mirault, Laurent Guibaud, Sylvie Fraitag, Sato Magassa, Antoine Fraissenon, Caroline Chopinet, Guillaume Canaud, Christophe Legendre, Christine Broissand, Célia Chapelle, Lola Zerbib, J. Duong, Sophia Ladraa, Sophie Kaltenbach, Charles Bayard, Junna Yamaguchi, Florence Delestre, Véronique Soupre, and Rubina Cassaca

Subjects
Pathology, medicine.medical_specialty, business.industry, Somatic cell, General Medicine, Mice, Thiazoles, Lymphatic system, PIK3CA gene, medicine, Animals, Humans, In patient, Lymphatic malformations, business, neoplasms
Abstract

Lymphatic cystic malformations are rare genetic disorders mainly due to somatic gain-of-function mutations in the PIK3CA gene. These anomalies are frequently associated with pain, inflammatory flar...

Published
2021

14. Author response for 'Influenza vaccination and prognosis of COVID ‐19 in hospitalized patients with diabetes: Results from the CORONADO study'

Author

null A Diallo, null M Pichelin, null M Wargny, null P Gourdy, null JB Bonnet, null S Hadjadj, null B Cariou, null A Sultan, null F Galtier, null Matthieu Wargny, null Pascale Mahot, null Bertrand Cariou, null Samy Hadjadj, null Matthieu Pichelin, null Anne‐Laure Fournier‐Guilloux, null Nicolas Mauduit, null Edith Bigot‐Corbel, null Anne‐Sophie Boureau, null Laure Dekcer, null Audrey Ernould, null Claire Primot, null Anne Seguin, null Marielle Joliveau, null Sonia Pouvreau, null Chloé FOURNIER, null Jeremy Thureau, null Edith Fonteneau, null Pamela Hublain, null Chu Nantes, null Carole Agasse, null Mathilde DE Kergaradec, null Vincent Minville, null Fanny Vardon‐Bounes, null Guillaume Martin‐Blondel, null Pierre Gourdy, null Blandine Tramunt, null Marie‐Christine Turnin, null Hélène Hanaire, null Jean‐Michel Mansuy, null Didier Fabre, null Marie‐Blanche Arhainx, null Laurent Cazals, null Laure Combes, null Emmanuelle Lami, null Mallory Cianferani, null Bruno Megarbane, null Pierre Leroy, null Jean‐François Gautier, null Tiphaine Vidal‐Trecan, null Jean‐Pierre Riveline, null Jean‐Louis Laplanche, null Stéphane Mouly, null Louis Potier, null Ronan Roussel, null Malak Taher, null Yawa Abouleka, null Fetta Yaker, null Aurelie Carlier, null Anne Boutten, null Marilyne Hallot‐Feron, null Fadila Mourah, null Charles Thivolet, null Emilie Blond, null Muriel Rolland, null Josep Verdecho Mendez, null Marine Alexandre, null Julien Pottecher, null Emilie Richer, null Laurent Meyer, null Florina Luca, null Jean‐Marc Lessinger, null Thibault Bahougne, null Bruno Guerci, null Lisa Ludwig, null Siham Benzirar, null Catherine Malaplate, null Thierry Matton, null Julien Poissy, null Karine Faure, null Pierre Fontaine, null Florence Baudoux, null Anne Vambergue, null Jean David Pekar, null Marc Lambert, null Cécile Yelnik, null Amélie Bruandet, null Laurent Petit, null Didier Neau, null Vincent Rigalleau, null Annie Berard, null Amandine Galioot, null Remy Coudroy, null Arnaud Thille, null René Robert, null France Roblot‐Cazenave, null Blandine Rammaert, null Pierre Jean Saulnier, null Xavier Piguel, null Nesrine Benhenda, null Camille Husson, null Celine Olivier, null Florence Torremocha, null Mathilde Fraty, null Marie Flamen d'assigny, null Aurelie Miot, null Valentin Bossard, null Kada Klouche, null Alain Makinson, null Ariane Sultan, null Jean‐Baptiste Bonnet, null Vincent Foulongne, null Florence Galtier, null Cécile Aubron, null Séverine Ansart, null Véronique Kerlan, null Pascale Quiniou, null Jean‐Luc Carre, null Stéphane Quesnot, null Bruno Laviolle, null Carole Schwebel, null Olivier Epaulard, null Pierre‐Yves Benhamou, null Cécile Betry, null Anne‐Laure Borel, null Sandrine Lablanche, null Dorra Guergour, null Catherine Duclos, null Emmanuel Cosson, null Erwan Guyot, null Aurore Deniau, null Phucthutrang Nguyen, null Yves Reznik, null Michael Joubert, null Stéphane Allouche, null Lydia Guittet, null Steven Grange, null Manuel Etienne, null Gaëtan Prévost, null Valéry Brunel, null Jean‐Christophe Lagier, null Didier Raoult, null Anne Dutour, null Bénédicte Gaborit, null Sandrine Boulllu, null Patrice Darmon, null Adèle Lasbleiz, null Mathieu Cerino, null Fanny Romain, null Marie Houssays, null Jean Pierre Quenot, null Lionel Piroth, null Bruno Vergès, null Laurence Duvillard, null Bernard Bonnotte, null Alain Mercat, null Vincent Dubee, null Ingrid Allix, null Patrice Rodien, null Robin Dhersin, null Maylis Lebeault, null Wojciech Trzepizur, null Jocelyne Loison, null Antoine Brangier, null Pierre Asfar, null Pascal Reynier, null Françoise Larcher, null Françoise Joubaud, null Marie‐Rita Andreu, null Geoffrey Urbanski, null Laurent Hubert, null Cedric Annweiler, null Jean Dellamonica, null Johan Courjon, null Nicolas Chevalier, null Giulia Chinetti, null Magda Chafai, null Bruno Mourvillier, null Firouze Bani‐Sadr, null Sarra Barraud, null Brigitte Delemer, null Philippe Gillery, null Pascale Labedade, null Amélie Chabrol, null Alfred Penfornis, null Catherine Petit, null Coralie Amadou, null Maxime Adler, null Clément Dubost, null Pierre‐Louis Conan, null Lyse Bordier, null Franck Ceppa, null Cyril Garcia, null Mathilde Sollier, null Olivier Dupuy, null Sophie Laplance, null Olivier Billuart, null Marie Joseph Aroulanda, null Frédérique Olivier, null Florence Ayon, null Nathalie Wilhelm, null Loic Epelboin, null Nadia Sabbah, null Aurelie Charpin, null Pierre Squara, null Olivier Belliard, null Claude Dubois, null Michel Marre, null Johann Auchabie, null Roxane Courtois, null Thierry Duriez, null Tiphaine Mergey, null Laura Vallee, null Laetitia Seguin, null Abdallah Al‐Salameh, null Jean‐Philippe Lanoix, null Sandrine Soriot‐Thomas, null Anne‐Marie Bourgeois‐Descouls, null Rachel Desailloud, null Natacha Germain, null Bogdan Galusca, null Gwenaelle Belleton, null Nesrine Marouani, null Delia Palaghiu, null Amira Hammour, null Fernando Berdaguer, null Thimothée Klopfenstein, null Hajer Zayet, null Patrice Winiszewski, null Marie Zanusso, null Pauline Garnier, null Ingrid Julier, null Karim Hamzaoui, null Sophie Marty‐Gres, null Tarik Sadki, null Lucile Cadot, null Jean‐Louis Dubost, null Céline Gonfroy, null Catherine Campinos, null Pascale Martres, null Marie Pierre Coulhon, null Nicolas Allou, null Marwa Bachir, null Stella Hoang, null Candice Kembellec, null Olivia Suply, null Fatima Kharcha, null Anne‐Claire Devouge, null Anna Flaus‐Furmanuk, null Isabelle Madeline, null Vincent Ehinger, null Sophie Bastard, null Loic Raffray, null Frederic Renou, null Aude Bojarski, null Caroline Paul, null Karine Borsu, null Angelique Gorlin, null Servane Bernardo, null Carole Truong Ut, null Stephane Renaud, null Antoine Vignoles, null Emilie Foch, null Laurie Masse, null Hubert Grand, null Helene Ferrand, null Christelle Raffaitin‐Cardin, null Hadjer Zellagui, null Celine Castang‐Brachet, null Frederique Boury, null Ana Alvarez Tena, null Isabelle Moura, null Pierre Kalfon, null Juliana Darasteanu, null Arnaud Monier, null Pascal Foucault, null Alexandra Depuille, null Stéphanie Laugier‐Robiolle, null Patrick Caneiro, null Maud Basso, null Etienne Larger, null Samir Bouam, null Wahiba Benzenati, null Leila Ait Bachir, null Camille Cussac Pillegand, null Marc Vasse, null Christophe Michard, null Nathanaëlle Montanier, null Luc Millot, null Françoise Crepet, null Danielle Ratsimba, null Kevin Bouiller, null Sophie Borot, null Isabelle Bruckert, null Annie Clergeot, null Franck Schillo, null Dorothée Vignes, null Muriel Bourgeon‐Ghittori, null Hamoud Lachgar, null Claire Lambert DE Cursay, null Stéphane Levante, null Jean Charles Auregan, null Antoine Merlet, null Cécile Zaragoza, null Gwénaëlle Arnault, null Anne‐Gaëlle Loupp, null Olivier Lesieur, null Mariam Roncato‐Saberan, null Didier Gouet, null Romain Lemarie, null Hong_an Allano, null Emmanuel Vivier, null Caroline Pariset, null Cédric Luyton, null Lucien Marchand, null Fanny Doroszewski, null Matthieu Pecquet, null Laurent Perard, null Sylvie Vuillermoz‐Blas, null Nicolas Kacki, null Patricia Charrier, null Amélie Ducet‐Boiffard, null Françoise Desroys Roure, null Olivier Bourron, null Dominique Bonnefont‐Rousselot, null Suzanne Laroche, null Franck Phan, null Agnès Hartemann, null Cyrielle Caussy, null Emmanuel Disse, null Claude Guerin, null Thomas Perpoint, null Philippe Moulin, null Régine Cartier, null Geoffroy Hariri, null Dorothée Chopin, null Camille Vatier, null Nathalie Bourcigaux, null Emmanuelle Chaigneau, null Sophie Christin‐Maitre, null Bruno Donadille, null Bruno Feve, null Sophie Lamothe, null Julie Sarfati, null Pascal Pernet, null Anne Chambon, null Delphine Demarsy, null Hugo Campagne, null Françoise Latil‐Plat, null Monica Berne, null Marilyne Grinand, null Marion Touzet, null Aydrey Zabulon, null Jocelyne Craspag, null Catherine Ledoux, null Cedric Contaret, null Blandine Janand‐Delenne, null Anaïs Giraud, null Marie Lou Lacrimini, null Joëlle Arrivie, null Deborah Ancelle, null Carine Guillois, null Bénédicte Fremy, null Amina Chaalal, null Gaëlle Barrande, null Anne Dorange, null Eglantine Rouanet, null Dominique Seret‐Begue, null Audrey Saoud, null Anne‐Marie Guedj, null Nathalie Bedos, null Fritz‐Line Velayoudom, null Marie Dumas, null Benoite Gonda, null Christine Coffin, null Stéphanie Gibiat, null Myriam Lungo, null Chantal Bully, null Pierre Serusclat, null Stella Bully, null Patricia Carre, null Jean‐Philippe Leberre, null Carlos Elkhoury, null Marine Thieux, null Laetitia Paradisi‐Prieur, and null CORONADO investigators

Subjects
Vaccination, Pediatrics, medicine.medical_specialty, Coronavirus disease 2019 (COVID-19), business.industry, Hospitalized patients, Diabetes mellitus, Medicine, business, medicine.disease
Published
2021

15. Clinical significance of anti-Ro52 (TRIM21) antibodies in adult patients with connective tissue diseases

Author

Eric Hachulla, Thomas Moulinet, S. Revuz, Roland Jaussaud, Sylvain Dubucquoi, Amandine Luc, Benjamin Lopez, Paul Decker, Bernard Bonnotte, Daniela Lakomy, Marcelo De Carvalho Bittencourt, Ingénierie Moléculaire et Physiopathologie Articulaire (IMoPA), and Université de Lorraine (UL)-Centre National de la Recherche Scientifique (CNRS)

Subjects
Adult, medicine.medical_specialty, genetic processes, Connective tissue, Antisynthetase syndrome, 030204 cardiovascular system & hematology, environment and public health, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Internal Medicine, medicine, Humans, Clinical significance, 030212 general & internal medicine, Connective Tissue Diseases, ComputingMilieux_MISCELLANEOUS, Autoantibodies, Retrospective Studies, biology, Adult patients, Myositis, business.industry, fungi, Interstitial lung disease, medicine.disease, Connective tissue disease, 3. Good health, enzymes and coenzymes (carbohydrates), medicine.anatomical_structure, Ribonucleoproteins, health occupations, biology.protein, [SDV.IMM]Life Sciences [q-bio]/Immunology, CTD, Antibody, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract

Objectives Clinical significance of anti-Ro52 antibodies in connective tissue diseases (CTD) is controversial. Anti-Ro52 antibodies might be associated with a more severe CTD phenotype, especially interstitial lung disease (ILD). The aims of this study were to evaluate ILD prevalence and severity, the prevalence of micro- or macroangiopathy and CTD-associated cancers in CTD with anti-Ro52 antibodies. Methods CTD patients with anti-Ro52 antibody screening by immunoblot at diagnosis were enrolled. Two groups were retrospectively formed according to the presence of anti-Ro52 antibodies with an unbiased 1:1 matching on CTD types. Unsupervised multiple correspondence analysis and hierarchical clustering analysis were used to aggregate anti-Ro52 positive patients in subgroups. Results 408 CTD patients were included. Anti-Ro52 antibodies were detected in 33 % of CTD patients. Anti-Ro52 antibodies were associated with ILD at CTD diagnosis (47.8% vs. 23.0%, OR 3.3 95% IC 1.4 to 8.0, p = 0.008), even after adjusting for the presence of anti-Ro60 antibodies, especially in patients with antisynthetase syndrome, primary Sjogren syndrome and systemic sclerosis. Micro- or macroangiopathy was more frequent in anti-Ro52 positive CTD patients (18.6% vs. 9.7%, p = 0.02) and CTD patients with anti-Ro52 antibodies experienced more frequent relapses and required more immunosuppressive drugs. Clusters 4 and 5 identified anti-Ro52 positive CTD patients with severe ILD and with clinical features of systemic sclerosis or antisynthetase syndrome respectively. Conclusions We found that anti-Ro52 antibodies were independently associated with ILD in CTD patients irrespective of CTD type. Anti-Ro52 antibodies could be associated with severity and a more relapsing disease course in CTD patients.

Published
2021

16. Off-label use of biologics for the treatment of refractory and/or relapsing granulomatosis with polyangiitis

Author

Amélie Servettaz, Philippe Guilpain, Grégory Pugnet, Fleur Cohen-Aubart, Bernard Bonnotte, Cécile-Audrey Durel, L Terrier, M. Hamidou, Vincent Poindron, Xavier Puéchal, C. Mettler, L. Guillevin, Jean-Christophe Lega, François Lifermann, and V. Le Guern

Subjects
medicine.medical_specialty, Biological Products, business.industry, Abatacept, Granulomatosis with Polyangiitis, Retrospective cohort study, Off-Label Use, Off-label use, medicine.disease, Gastroenterology, Infliximab, Treatment Outcome, Refractory, Prednisone, Internal medicine, Internal Medicine, medicine, Adalimumab, Humans, Tumor Necrosis Factor Inhibitors, Granulomatosis with polyangiitis, business, medicine.drug, Retrospective Studies
Abstract

Objective To describe the efficacy and safety of off-label use of biologics for refractory and/or relapsing granulomatosis with polyangiitis (GPA). Methods We conducted a French retrospective study including GPA patients who received off-label biologics for refractory and/or relapsing disease after failure of conventional immunosuppressive regimens. Results Among 26 patients included, 18 received infliximab (IFX), 2 adalimumab (ADA) and 6 abatacept (ABA). Biologics were initiated in median as 4th-line therapy (IQR 3–6) for relapsing and/or refractory disease in 23 (88%) and/or significant glucocorticoid-dependency in 8 cases (31%). At biologics initiation, median (IQR) BVAS and prednisone dose in anti- TNF-α and ABA recipients were 7 (3–8) and 2 (1–6), and 20 (13–30) mg/day and 20 (15–25) mg/day, respectively. Clinical manifestations requiring biologics were mainly pulmonary and ENT manifestations in 58% each. Anti-TNF-α and ABA were continued for a median duration of 8 months (IQR 6–13) and 11 months (IQR 6–18) respectively. Anti-TNF-α recipients showed remission, partial response and treatment failure in 10%, 30% and 60% at 6 months, and 25%, 20% and 55% at 12 months, respectively. ABA recipients showed remission, partial response and treatment failure in 17%, 33% and 50% at 6 months and 17%, 33% and 50% at 12 months. One patient treated with IFX experienced life-threatening reaction while one patient treated with ABA experienced a severe infection. Conclusion This real-life study suggests that off-label use of anti-TNF-α and abatacept shows efficacy in less than 50% of refractory and/or relapsing GPA.

Published
2021

17. Adrenal Insufficiency Revealing a Bilateral Adrenal Hemorrhage-Adrenal Infarction Related to Antiphospholipid Syndrome

Author

Maxime Samson, Bernard Bonnotte, Jean-Michel Petit, Anne-Claire Billet, Sylvain Audia, and Olivier Chevallier

Subjects
medicine.medical_specialty, business.industry, Hemorrhage, General Medicine, Middle Aged, Antiphospholipid Syndrome, medicine.disease, Infarction, Antiphospholipid syndrome, Mineralocorticoids, Internal medicine, Adrenal Glands, Adrenal insufficiency, Cardiology, Humans, Medicine, Female, Adrenal infarction, Adrenal Hemorrhage, business, Glucocorticoids, Adrenal Insufficiency
Published
2022

18. Characteristics of Adult Patients with Idiopathic Retroperitoneal Fibrosis and Assessment of Risk of Relapse at Diagnosis

Author

Sébastien Humbert, Philip Bielefeld, Nadine Magy-Bertrand, Bastien Bouldoires, Bernard Bonnotte, Veronique Fournier, Jerome Razanamahery, and Gilles Blaison

Subjects
medicine.medical_specialty, medicine.medical_treatment, risk of relapse, lcsh:Medicine, 030204 cardiovascular system & hematology, Retroperitoneal fibrosis, Article, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, idiopathic retroperitoneal fibrosis, medicine, Relapse risk, Idiopathic Retroperitoneal Fibrosis, 030203 arthritis & rheumatology, Adult patients, business.industry, lcsh:R, General Medicine, medicine.disease, smoking cessation, Radiological weapon, Smoking cessation, Chronic renal failure, medicine.symptom, business, Infiltration (medical)
Abstract

Objectives: To compare adult patients’ characteristics suffering from idiopathic retroperitoneal fibrosis between “relapse-free” and relapsing patients at the diagnosis and identify factors associated with relapse at initial presentation. Methods: We conducted a retrospective multicentric study in four hospitals in Eastern France, from 1993 to 2020, of adult patients suffering from idiopathic retroperitoneal fibrosis. We analyzed clinical, biological, and radiological features at diagnosis and during a forty-month follow-up. Results: Of 47 patients suffering from retroperitoneal fibrosis, 21 patients had idiopathic retroperitoneal fibrosis. Among them, 13 experienced one or more relapses during follow-up. At diagnosis, clinical characteristics, relevant comorbidities, biological and radiological features were similar between groups. Smoking cessation seems associated with decreased relapse risk (p: 0.0624). A total of 8 patients developed chronic renal failure during follow-up. Ureteral infiltration at diagnosis was associated with evolution to chronic renal failure (p: 0.0091). Conclusion: No clinical, biological, or radiological features could predict relapse at retroperitoneal fibrosis diagnosis, but smoking cessation may prevent relapse.

Published
2021

19. Absence of Anti-Glomerular Basement Membrane Antibodies in 200 Patients With Systemic Lupus Erythematosus With or Without Lupus Nephritis: Results of the GOODLUPUS Study

Author

Bernard Bonnotte, P. Orquevaux, Bach Nga Pham, Zahir Amoura, S. Revuz, Delphine Giusti, Laurent Arnaud, Moustapha Dramé, Gilles Blaison, Marcelle Tonye Libyh, Thierry Martin, Hanns-Martin Lorenz, Nadine Magy-Bertrand, Grace Stockton-Bliard, Reinhard E. Voll, Amélie Servettaz, Grégory Gatouillat, Jan Chrusciel, Nellie Bourse Chalvon, Jean-Loup Pennaforte, Andreas Schwarting, Thierry Tabary, Oliver Hinschberger, Centre Hospitalier Universitaire de Reims (CHU Reims), Immuno-Régulation dans les Maladies Auto-Immunes Inflammatoires et le Cancer - EA 7509 (IRMAIC), and Université de Reims Champagne-Ardenne (URCA)

Subjects
Male, Anti-Glomerular Basement Membrane Disease, [SDV]Life Sciences [q-bio], Lupus nephritis, Aucun, urologic and male genital diseases, Severity of Illness Index, Gastroenterology, anti-glomerular basement membrane antibodies, 0302 clinical medicine, systemic lupus erythematosus, Lupus Erythematosus, Systemic, Immunology and Allergy, 030212 general & internal medicine, Original Research, medicine.diagnostic_test, biology, anti-GBM glomerulonephritis, Glomerular basement membrane, IIf, Middle Aged, 3. Good health, Titer, medicine.anatomical_structure, [SDV.IMM]Life Sciences [q-bio]/Immunology, Female, Antibody, Vasculitis, Adult, lcsh:Immunologic diseases. Allergy, medicine.medical_specialty, Immunology, 03 medical and health sciences, Antigen, Internal medicine, anti-GBM antibodies, medicine, Humans, Autoantibodies, Retrospective Studies, 030203 arthritis & rheumatology, lupus nephritis, business.industry, Goodpasture disease, medicine.disease, Case-Control Studies, Immunoassay, biology.protein, business, lcsh:RC581-607, Biomarkers
Abstract

IntroductionAnti-glomerular basement membrane (GBM) antibodies are pathogenic antibodies first detected in renal-limited anti-GBM disease and in Goodpasture disease, the latter characterized by rapidly progressive crescentic glomerulonephritis combined with intra-alveolar hemorrhage. Studies have suggested that anti-GBM antibody positivity may be of interest in lupus nephritis (LN). Moreover, severe anti-GBM vasculitis cases in patients with systemic lupus erythematosus (SLE) have been described in the literature, but few studies have assessed the incidence of anti-GBM antibodies in SLE patients.ObjectiveThe main study objective was to determine if positive anti-GBM antibodies were present in the serum of SLE patients with or withoutproliferativerenal damage and compared to a healthy control group.MethodologyThis retrospective study was performed on SLE patients’ sera from a Franco-German European biobank, developed between 2011 and 2014, from 17 hospital centers in the Haut-Rhin region. Patients were selected according to their renal involvement, and matched by age and gender. The serum from healthy voluntary blood donors was also tested. Anti-GBM were screened by fluorescence enzyme immunoassay (FEIA), and then by indirect immunofluorescence (IIF) in case of low reactivity detection (titer >6 U/ml).ResultsThe cohort was composed of 100 SLE patients withproliferativeLN (27% with class III, 67% with class IV, and 6% with class V), compared to 100 SLE patients without LN and 100 controls. Patients were mostly Caucasian and met the ACR 1997 criteria and/or the SLICC 2012 criteria. Among the 300 tested sera, no significant levels of anti-GBM antibodies were detected (>10 U/ml) by the automated technique, three sera were found “ambivalent” (>7 U/ml): one in the SLE with LN group and two in the SLE without LN group. Subsequent IIF assays did not detect anti-GBM antibodies.ConclusionAnti-GBM antibodies were not detected in the serum of Caucasian patients with SLE, even in case of renal involvement, a situation favoring the antigenic exposure of glomerular basement membranes. Our results reaffirm the central role of anti-GBM antibodies as a specific diagnostic biomarker for Goodpasture vasculitis and therefore confirm that anti-GBM antibody must not be carried out in patients with SLE (with or without LN) in the absence of disease-suggestive symptoms.

Published
2020

21. Evans’ Syndrome: From Diagnosis to Treatment

Author

Marc Michel, Bernard Bonnotte, Sylvain Audia, Natacha Grienay, and Morgane Mounier

Subjects
Pediatrics, medicine.medical_specialty, Evans syndrome, medicine.medical_treatment, Splenectomy, lcsh:Medicine, Disease, Review, 03 medical and health sciences, 0302 clinical medicine, hemic and lymphatic diseases, Medicine, Cytopenia, business.industry, Myelodysplastic syndromes, lcsh:R, General Medicine, medicine.disease, Evans’ syndrome, immune thrombocytopenia, 030220 oncology & carcinogenesis, Concomitant, Autoimmune neutropenia, Rituximab, autoimmune haemolytic anaemia, business, 030215 immunology, medicine.drug
Abstract

Evans’ syndrome (ES) is defined as the concomitant or sequential association of warm auto-immune haemolytic anaemia (AIHA) with immune thrombocytopenia (ITP), and less frequently autoimmune neutropenia. ES is a rare situation that represents up to 7% of AIHA and around 2% of ITP. When AIHA and ITP occurred concomitantly, the diagnosis procedure must rule out differential diagnoses such as thrombotic microangiopathies, anaemia due to bleedings complicating ITP, vitamin deficiencies, myelodysplastic syndromes, paroxysmal nocturnal haemoglobinuria, or specific conditions like HELLP when occurring during pregnancy. As for isolated auto-immune cytopenia (AIC), the determination of the primary or secondary nature of ES is important. Indeed, the association of ES with other diseases such as haematological malignancies, systemic lupus erythematosus, infections, or primary immune deficiencies can interfere with its management or alter its prognosis. Due to the rarity of the disease, the treatment of ES is mostly extrapolated from what is recommended for isolated AIC and mostly relies on corticosteroids, rituximab, splenectomy, and supportive therapies. The place for thrombopoietin receptor agonists, erythropoietin, immunosuppressants, haematopoietic cell transplantation, and thromboprophylaxis is also discussed in this review. Despite continuous progress in the management of AIC and a gradual increase in ES survival, the mortality due to ES remains higher than the ones of isolated AIC, supporting the need for an improvement in ES management.

Published
2020

22. Ustekinumab For the Treatment of Giant Cell Arteritis: Comment on the Article by Matza et al

Author

Bernard Bonnotte and Maxime Samson

Subjects
030203 arthritis & rheumatology, medicine.medical_specialty, medicine.diagnostic_test, business.industry, Giant Cell Arteritis, medicine.disease, Gastroenterology, 03 medical and health sciences, chemistry.chemical_compound, Giant cell arteritis, 0302 clinical medicine, Tocilizumab, Rheumatology, chemistry, Prednisone, Internal medicine, Erythrocyte sedimentation rate, Ustekinumab, medicine, Clinical endpoint, Humans, In patient, business, medicine.drug
Abstract

We read with great interest the paper of Matza et al. (1) reporting on their prospective, open-label, single-arm, single-centre trial which was designed to evaluate the efficacy and safety of ustekinumab (UST) in combination with prednisone in patients with active GCA (ClinicalTrials.gov NCT02955147). The authors concluded that UST is an ineffective treatment for GCA since only 23% of the 13 patients enrolled achieved the primary endpoint (i.e. absence of relapse through week 52 and normalization of the erythrocyte sedimentation rate and C-reactive protein), which is inferior to previous results obtained in patients treated with tocilizumab (TCZ) (2).

Published
2020

23. Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Author

Bernhard Hellmich, Maxime Samson, Luc Mouthon, Juliane Mahrhold, Francesco Locatelli, Nils Venhoff, C. Kroegel, Matthias Papo, Thomas Barba, Thomas Neumann, M.L. Urban, Matthieu Groh, Giulia Cassone, Carlo Salvarani, Loïc Guillevin, Xavier Puéchal, Benjamin Terrier, Bernard Bonnotte, Chiara Marvisi, Vítor Teixeira, Michele Iudici, Augusto Vaglio, Sara Monti, Arianna Troilo, Giacomo Emmi, Renato Alberto Sinico, Cécile-Audrey Durel, Jean-Emmanuel Kahn, David Jayne, Carlomaurizio Montecucco, Franco Schiavon, Jens Thiel, Benjamin Seeliger, Papo, M, Sinico, R, Teixeira, V, Venhoff, N, Urban, M, Iudici, M, Mahrhold, J, Locatelli, F, Cassone, G, Schiavon, F, Seeliger, B, Neumann, T, Kroegel, C, Groh, M, Marvisi, C, Samson, M, Barba, T, Jayne, D, Troilo, A, Thiel, J, Hellmich, B, Monti, S, Montecucco, C, Salvarani, C, Kahn, J, Bonnotte, B, Durel, C, Puéchal, X, Mouthon, L, Guillevin, L, Emmi, G, Vaglio, A, and Terrier, B

Subjects
Adult, Male, Vasculitis, medicine.medical_specialty, 030204 cardiovascular system & hematology, Churg-Strauss Syndrome, urologic and male genital diseases, Gastroenterology, Antibodies, Antineutrophil Cytoplasmic, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, immune system diseases, Internal medicine, Eosinophilic, Humans, Medicine, Pharmacology (medical), cardiovascular diseases, ANCA, Churg-Strauss, vasculitis, skin and connective tissue diseases, Churg strauss, Aged, Retrospective Studies, Asthma, 030203 arthritis & rheumatology, ddc:616, business.industry, Hazard ratio, Granulomatosis with Polyangiitis, Middle Aged, Eosinophil, medicine.disease, Dermatology, respiratory tract diseases, Peripheral neuropathy, medicine.anatomical_structure, Churg-Strau, vasculiti, Cohort, Female, business, Granulomatosis with polyangiitis, Immunosuppressive Agents
Abstract

Objectives Only a third of patients with eosinophilic granulomatosis with polyangiitis (EGPA) are ANCA-positive, mainly directed against MPO. ANCA directed against PR3 are rarely found in EGPA. We aimed to examine the significance of PR3-ANCA in EGPA. Methods We set up a retrospective European multicentre cohort including 845 patients. Baseline characteristics and outcomes were analysed and compared according to ANCA status. Results ANCA status was available for 734 patients: 508 (69.2%) ANCA-negative, 210 (28.6%) MPO-ANCA and 16 (2.2%) PR3-ANCA. At baseline, PR3-ANCA patients, compared with those with MPO-ANCA and ANCA-negative, less frequently had active asthma (69% vs 91% and 93%, P = 0.003, respectively) and peripheral neuropathy (31% vs 71% and 47%, P Conclusion PR3-ANCA EGPA patients differ from those with MPO-ANCA and negative ANCA, and share clinical features with granulomatosis with polyangiitis. This suggests that PR3-ANCA EGPA could be a particular form of PR3-ANCA-associated vasculitis.

Published
2020

24. CT analysis of the aorta in giant-cell arteritis: a case-control study

Author

Hervé Devilliers, Olivier Chevallier, Frédéric Ricolfi, Bernard Bonnotte, Paul Ornetti, Serge Aho-Glélé, Romaric Loffroy, Pierre Emmanuel Berthod, Maxime Samson, Department vascular, oncologic ans interventional radiology (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Service de radiologie et d'Imagerie médicale diagnostique et thérapeutique (CHU de Dijon), Service Biostatistiques et Informatique Médicale (CHU de Dijon) (DIM), Service de Rhumatologie (CHU de Dijon), Service de Médecine Interne (SOC 1 et SOC 2) [CHU de Dijon], Service de médecine interne et maladies systémiques (SOC 2) [CHU de Dijon], Service de neuroradiologie, imagerie des urgences [CHU de Dijon], Service de médecine interne et immunologie clinique (SOC 1) [CHU de Dijon], The Johns Hopkins University School of Medicine, Johns Hopkins University (JHU), Laboratoire d'Electronique, d'Informatique et d'Image [EA 7508] (Le2i), Université de Technologie de Belfort-Montbeliard (UTBM)-Université de Bourgogne (UB)-École Nationale Supérieure d'Arts et Métiers (ENSAM), Arts et Métiers Sciences et Technologies, HESAM Université (HESAM)-HESAM Université (HESAM)-Arts et Métiers Sciences et Technologies, and HESAM Université (HESAM)-HESAM Université (HESAM)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Centre National de la Recherche Scientifique (CNRS)

Subjects
Male, [SDV.IB.IMA]Life Sciences [q-bio]/Bioengineering/Imaging, Cardiovascular-Disease, 030204 cardiovascular system & hematology, Temporal Arteritis, 0302 clinical medicine, immune system diseases, CT angiography Term-Follow-Up, Thoracic aorta, skin and connective tissue diseases, Aorta, Neuroradiology, General Medicine, Classification, cardiovascular system, Female, [SDV.IB]Life Sciences [q-bio]/Bioengineering, Radiology, Vasculitis, Risk, medicine.medical_specialty, Giant Cell Arteritis, [SDV.IB.MN]Life Sciences [q-bio]/Bioengineering/Nuclear medicine, Sensitivity and Specificity, Polymyalgia rheumatica, 03 medical and health sciences, medicine.artery, [INFO.INFO-IM]Computer Science [cs]/Medical Imaging, medicine, Humans, Radiology, Nuclear Medicine and imaging, cardiovascular diseases, Arteritis, Aged, Inflammation, 030203 arthritis & rheumatology, Aortitis, business.industry, Atherosclerosis, Polymyalgia-Rheumatica, medicine.disease, Giant cell aortitis, Giant cell arteritis, Atheroma, ROC Curve, Case-Control Studies, Involvement, Tomography, X-Ray Computed, business
Abstract

International audience; Giant cell arteritis (GCA) is a large-vessel vasculitis whose diagnosis is confirmed by temporal artery biopsy. However, involvement of large vessels, especially the aorta, can be shown by imaging, which plays an increasing role in GCA diagnosis. The threshold above which aortic wall thickening, as measured by computed tomography (CT), is considered pathological is controversial, with values ranging from 2 to 3 mm. This study assessed aortic morphology by CT scan and its diagnostic value in GCA. Altogether, 174 patients were included (64 with GCA, 43 with polymyalgia rheumatica and 67 controls). All patients had a CT scan at diagnosis or at inclusion for controls. Aortic wall thickness, aortic diameter and scores for atheroma were measured. Assessor was blinded to each patient's group. Aortic diameters and atheroma scores were similar between groups. Aortic wall thickness was greater in the GCA group, even after the exclusion of GCA patients with aortic wall thickness >= 3 mm. The receiver operating characteristic (ROC) curve showed that a wall thickness of 2.2 mm was the optimal threshold to diagnose GCA (sensitivity, 67%; specificity, 98%). Measuring aortic wall thickness by CT scan is effective to diagnose GCA. The optimal threshold to regard aortic wall thickening as pathological was >= 2.2 mm. aEuro cent Imaging, including CT scan, plays an increasing role in GCA diagnosis aEuro cent CT measurement of aortic wall thickness is useful to diagnose GCA aEuro cent A 2.2-mm threshold allows the diagnosis of thickened aortic wall in GCA.

Published
2018

25. Diagnosis of hyperferritinemia in routine clinical practice

Author

Bernard Lorcerie, Sylvain Audia, Bernard Bonnotte, Nicolas Falvo, Vanessa Leguy-Seguin, Maxime Samson, Aurélie Millière, and Sabine Berthier

Subjects
0301 basic medicine, Iron metabolism disorder, Pediatrics, medicine.medical_specialty, 03 medical and health sciences, 0302 clinical medicine, Humans, Medicine, Routine clinical practice, biology, Diagnostic Tests, Routine, business.industry, Transferrin saturation, General Medicine, medicine.disease, Iron Metabolism Disorders, Surgery, Ferritin, Hepatic Iron Concentration, 030104 developmental biology, Chronic dialysis, 030220 oncology & carcinogenesis, Hereditary hemochromatosis, Ferritins, biology.protein, Metabolic syndrome, business
Abstract

The discovery of hyperferritinemia is often fortuitous, revealed in results from a laboratory screening or follow-up test. The aim of the diagnostic procedure is therefore to identify its cause and to identify or rule out hepatic iron overload, in a three-stage process. In the first step, clinical findings and several simple laboratory tests are sufficient to detect four of the most frequent causes of high ferritin concentrations: alcoholism, inflammatory syndrome, cytolysis, and metabolic syndrome. None of these causes is associated with substantial hepatic iron overload. If transferrin saturation is high (> 50%), hereditary hemochromatosis will be considered in priority. In the second phase, rarer diseases will be sought. Among them, only chronic hematologic diseases (acquired or congenital) and excessive iron intake or infusions (patients on chronic dialysis and high-level athletes) are at risk of iron overload. In the third stage, if a doubt persists about the cause or if the ferritin concentration is very high or continues to rise, it is essential to verify the hepatic iron concentration to rule out overload. The principal examination to guide diagnosis and treatment is hepatic MRI to assess its iron concentration. It is essential to remember that more than 40% of patients with hyperferritinemia have several causes simultaneously present.

Published
2017

26. De la physiopathologie de l’artérite à cellules géantes aux nouvelles cibles thérapeutiques

Author

Bernard Bonnotte and Maxime Samson

Subjects
030203 arthritis & rheumatology, 0301 basic medicine, Gynecology, medicine.medical_specialty, Pathology, business.industry, Gastroenterology, medicine.disease, 03 medical and health sciences, Giant cell arteritis, 030104 developmental biology, 0302 clinical medicine, Internal Medicine, medicine, business
Abstract

Resume L’arterite a cellules geantes (ACG) est la plus frequente des vascularites de l’adulte. Il s’agit d’une vascularite granulomateuse, affectant les vaisseaux de gros calibre et survenant chez les patients de plus de 50 ans. La pierre angulaire du traitement de l’ACG reste a l’heure actuelle la corticotherapie, debutee entre 0,7 et 1 mg/kg/j en fonction de l’existence de complication(s) ischemique(s) et poursuivie en moyenne 18 mois. Ce traitement est tres efficace mais le sevrage progressif des corticoides est associe a la survenue de rechutes. De plus, cette corticotherapie au long cours est responsable de nombreuses complications iatrogenes. La corticotherapie n’est donc pas le traitement ideal de l’ACG et des strategies d’epargne en corticoides doivent etre developpees. La physiopathologie de l’ACG n’est pas completement connue mais des progres majeurs ont ete realises ces dernieres annees. Si son facteur declenchant, suspecte etre d’origine infectieuse, reste pour le moment inconnu, les mecanismes (en particulier immunologiques) conduisant a l’inflammation granulomateuse de la paroi vasculaire et a son remodelage progressif, source de complications ischemiques, sont de mieux en mieux connus et ont permis l’emergence de nouvelles cibles therapeutiques, en particulier le blocage de l’activation du lymphocyte T ou l’inhibition de la signalisation de l’interleukine-6 (IL-6), de l’IL-12/23 ou de l’IL-1β.

Published
2017

27. 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

28. Le tocilizumab signifie-t-il la fin de la corticothérapie au cours de l’artérite à cellules géantes ?

Author

Bernard Bonnotte and Maxime Samson

Subjects
030203 arthritis & rheumatology, Pathology, medicine.medical_specialty, biology, business.industry, Gastroenterology, medicine.disease, 03 medical and health sciences, Giant cell arteritis, chemistry.chemical_compound, 0302 clinical medicine, Tocilizumab, chemistry, Monoclonal, Internal Medicine, medicine, biology.protein, 030212 general & internal medicine, Antibody, business
Published
2018

29. Traitement de l’artérite à cellules géantes

Author

T. Ghesquiere, Bernard Bonnotte, Maxime Samson, Hélène Greigert, Service de médecine interne et immunologie clinique (SOC 1) [CHU de Dijon], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Interactions hôte-greffon-tumeur, ingénierie cellulaire et génique - UFC (UMR INSERM 1098) (RIGHT), 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]), CCSD, Accord Elsevier, 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), and Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC)

Subjects
030203 arthritis & rheumatology, Gynecology, [SDV] Life Sciences [q-bio], 03 medical and health sciences, medicine.medical_specialty, 0302 clinical medicine, business.industry, [SDV]Life Sciences [q-bio], Medicine, 030212 general & internal medicine, General Medicine, business, 3. Good health
Abstract

Points essentiels En 2019, la corticotherapie reste le traitement de reference de l’arterite a cellules geantes (ACG) mais a condition de respecter des valeurs seuils concernant la decroissance des doses : 15 a 20 mg/j a 3 mois, 10 mg/j a 6 mois, 5 mg/j a 9–12 mois et sevrage en 12 a 18 mois. En cas de corticodependance a plus de 7,5 mg/j de prednisone ou d’intolerance de la corticotherapie, un traitement d’epargne en corticoides doit etre instaure, en premiere intention, methotrexate ou tocilizumab. Le choix d’un traitement d’epargne en corticoides doit prendre en consideration les caracteristiques individuelles du patient, les donnees d’efficacite disponibles concernant ce traitement, son cout et les modalites de suivi des patients. C’est pour cela que, sauf exception, nous utilisons d’abord le methotrexate et ensuite le tocilizumab. La prevention des evenements cardio-vasculaires est un element essentiel de la prise en charge des patients atteints d’ACG. Nous recommandons la prescription d’aspirine (75–100 mg/j) au cours du 1er mois de traitement et au long cours en cas de complication ischemique. Tout patient atteint d’ACG doit recevoir un traitement preventif de l’osteoporose selon les recommandations habituelles.

Published
2019

30. Association of antiphospholipid antibodies with active digital ulceration in systemic sclerosis

Author

Jean-Christophe Weber, Vincent Poindron, Jean-Loup Pennaforte, Gilles Blaison, Denis Wahl, C. Martinez, Nadine Magy-Bertrand, Philip Bielefeld, Pierre Kieffer, Bernard Bonnotte, Hervé Devilliers, Sabine Berthier, Thierry Martin, Laurent Arnaud, Mickael Martin, François Maurier, Médecine interne [ Poitiers], Centre hospitalier universitaire de Poitiers (CHU Poitiers), Médecine interne [Hôpitaux civils de Colmar], Hôpitaux Civils de Colmar, Service de rhumatologie [Strasbourg], CHU Strasbourg-Hôpital de Hautepierre [Strasbourg], Centre National de Référence pour les Maladies Auto-immunes Rares [CHU Strasbourg] (RESO), CHU Strasbourg, Médecine interne [CHU Strasbourg], Département de Médecine Interne et Immunologie Clinique (DMIIC - STRASBOURG), Médecine interne [CH Emile Muller, Mulhouse], 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 et immunologie clinique (SOC 1) [CHU de Dijon], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Service de Médecine Interne et Vasculaire [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Centre régional de compétence des Maladies systémiques et auto-immunes rares de l'adulte et Maladies vasculaires rares, Faculté de Médecine [Nancy], Université de Lorraine (UL), Service de Médecine Interne [CHR Metz-Thionville], Centre hospitalier régional Metz-Thionville (CHR Metz-Thionville), Service de Rhumatologie [Reims], Centre Hospitalier Universitaire de Reims (CHU Reims), Service de médecine interne (Med Int - BESANCON), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), and WEBER, JEAN-CHRISTOPHE

Subjects
Male, Raynaud’s phenomenon, 0302 clinical medicine, Risk Factors, Informed consent, Immunology and Allergy, Medicine, 030212 general & internal medicine, skin and connective tissue diseases, ComputingMilieux_MISCELLANEOUS, Gangrene, [SDV.MHEP.RSOA] Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, integumentary system, biology, antiphospholipid antibodies, Interstitial lung disease, Middle Aged, Pathophysiology, 3. Good health, [SDV.MHEP.RSOA]Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, Antibodies, Antiphospholipid, [SDV.IMM]Life Sciences [q-bio]/Immunology, Female, Disease Susceptibility, Antibody, Adult, medicine.medical_specialty, [SDV.IMM] Life Sciences [q-bio]/Immunology, Immunology, digital ulcer, Systemic Sclerosis, Fingers, 03 medical and health sciences, Rheumatology, Antiphospholipid syndrome, Internal medicine, Skin Ulcer, Humans, Aged, Autoantibodies, 030203 arthritis & rheumatology, Autoimmune disease, Scleroderma, Systemic, business.industry, medicine.disease, Cross-Sectional Studies, biology.protein, business, antiphospholipid syndrome, Biomarkers
Abstract

Key messages #### What is already known about this subject? #### What does this study add? #### How might this impact on clinical practice? Raynaud’s phenomenon (RP) in systemic sclerosis (SSc) can be severe with active digital ulceration (ADU) and gangrene. RP physiopathology includes early endothelial cell injury, vascular dysfunction and sometimes microvascular thrombosis.1 Antiphospholipid antibodies (aPL) activate endothelial cells and platelets by complexes of beta-2 glycoprotein 1 (β2GP1) and anti-β2GP12 and could therefore contribute to the initiation of and/or aggravate SSc-related RP. aPL prevalence seems increased in patients with SSc compared with controls,3 but aPL-associated clinical features are often contradictory. The aims of this study were to assess aPL and antiphospholipid syndrome (APS) prevalence in patients with SSc and their association with ADU. Consecutive patients aged more than 18 years, fulfilling the American College of Rheumatology/EULAR classification criteria for SSc4 and followed in seven French expert centres for autoimmune diseases labelled by the French national network for autoimmune disease care, were enrolled prospectively during 8 months. Patients with other associated autoimmune disease were excluded. All patients provided written informed consent. SSc subtype was classified based on LeRoy and Medsger’s criteria,5 and skin involvement was assessed according to the modified Rodnan skin score (mRSS).6 Interstitial lung disease …

Published
2019

31. Characteristics, risk factors and management of venous thromboembolism in immune thrombocytopenia: a retrospective multicentre study

Author

Bernard Bonnotte, Marc Ruivard, Marc Michel, Bertrand Godeau, Sylvain Audia, Guillaume Le Guenno, Mehdi Khellaf, Romain Guieze, Role of intra-Clonal Heterogeneity and Leukemic environment in ThErapy Resistance of chronic leukemias (CHELTER), and Université Clermont Auvergne [2017-2020] (UCA [2017-2020])

Subjects
Adult, Male, medicine.medical_specialty, Adolescent, Databases, Factual, medicine.drug_class, Hemorrhage, 030204 cardiovascular system & hematology, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Recurrence, Risk Factors, hemic and lymphatic diseases, Internal medicine, Internal Medicine, medicine, Humans, Platelet, cardiovascular diseases, 030212 general & internal medicine, ComputingMilieux_MISCELLANEOUS, Aged, Retrospective Studies, Aged, 80 and over, Purpura, Thrombocytopenic, Idiopathic, Platelet Count, business.industry, Anticoagulant, Anticoagulants, Cancer, Retrospective cohort study, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, Venous Thromboembolism, Middle Aged, equipment and supplies, medicine.disease, Thrombosis, Thrombocytopenic purpura, 3. Good health, Female, France, Complication, business, Venous thromboembolism
Abstract

BACKGROUND An increased risk of thrombosis has been reported in immune thrombocytopenic purpura (ITP), but the characteristics, risk factors of occurrence, recurrence and management of venous thromboembolic events (VTE) have been poorly investigated. AIMS To describe VTE and ITP characteristics, distribution of VTE risk factors and their impact on VTE features and recurrence. METHODS A retrospective study of patients with ITP and VTE registered in databases of three reference French centres of ITP. RESULTS Among 49 patients, 66 VTE were recorded. The platelet count at the time of the first VTE was

Published
2019

32. SAT0239 PREDICTION OF LONG-TERM EVOLUTIONARY PROFILES IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURG–STRAUSS) BASED ON BASELINE AND FOLLOW-UP CHARACTERISTICS

Author

Thomas Barba, Cécile-Audrey Durel, Jean-Emmanuel Kahn, Benjamin Seeliger, Claus Kroegel, Loïc Guillevin, Bernard Bonnotte, Matthieu Groh, Augusto Vaglio, Sara Monti, David Jayne, Carlomaurizio Montecucco, Bernhard Hellmich, Franco Schiavon, Thomas Neumann, Vitor H. Teixeira, Chiara Marvisi, Luc Mouthon, Maxime Samson, Juliane Mahrhold, Xavier Puéchal, Carlo Salvarani, Renato Alberto Sinico, Giulia Cassone, Matthias Papo, Benjamin Terrier, M.L. Urban, and Giacomo Emmi

Subjects
Pediatrics, medicine.medical_specialty, business.industry, Complete remission, medicine.disease, Prednisone, Eosinophilic, Cohort, medicine, Granulomatosis with polyangiitis, business, Vasculitis, Churg strauss, medicine.drug, Asthma
Abstract

Background Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg–Strauss) is a small-vessel necrotizing vasculitis characterized by blood and tissue eosinophilia and asthma. Glucocorticoids (GCs) control the disease, but GC-dependence is frequent. Evolving concepts distinguish vasculitis-related symptoms from asthma and/or ENT manifestations. That distinction has become even more important since the development of B-cell and eosinophil-targeted therapies. Objectives This study aimed to describe and identify characteristics predicting long-term EGPA outcomes. Methods We set up a multicenter European cohort that included 636 EGPA patients. Based on recent consensus, we distinguished 4 EGPA-evolutionary profiles: GC-dependent asthma and/or ENT manifestations (requiring prednisone >7.5 mg/d), ≥1 vasculitis relapse(s) (excluding asthma and/or ENT flares), both phenotypes, and complete remission (no GC-dependent asthma/ENT signs, no vasculitis relapse and prednisone Results After median follow-up of 63 (IQR 30-110) months, 35.8% had GC-dependent asthma and/or ENT manifestations, 12.9% had ≥1 vasculitis relapse(s), 14.3% had both phenotypes, 14.6% were in complete remission, 14.4% were in partial remission and 7.8% had not reach remission. Patients with GC-dependent asthma/ENT manifestations were younger at diagnosis (p Patients with vasculitis relapse(s) had more frequently neurological manifestations at diagnosis (p=0.002) and MPO-ANCA positivity (p Finally, patients in complete remission were older (p Conclusion EGPA seems to evolve toward distinct phenotypic profiles, which could be identified using baseline and follow-up characteristics. Early identification of those profiles could allow guided choices of the best therapeutic option. Disclosure of Interests Matthias Papo: None declared, Renato A. Sinico: None declared, Vitor Teixeira: None declared, Maria-Letizia Urban: None declared, Juliane Mahrhold: None declared, Sara Monti: None declared, Giulia Cassone: None declared, Franco Schiavon: None declared, Benjamin Seeliger: None declared, Thomas Neumann: None declared, Claus Kroegel: None declared, Matthieu Groh: None declared, Chiara Marvisi: None declared, Maxime Samson: None declared, Thomas Barba: None declared, David Jayne Grant/research support from: David Jayne has received research grants from Chemocentryx, GSK, Roche/Genentech and Sanofi-Genzyme. He has received consultancy fees from Astra-Zeneca, Boehringer-Ingelheim, Chemocentryx, Chugai, GSK, Infla-RX, Insmed and Takeda, Bernhard Hellmich Consultant for: Roche, Speakers bureau: Abbvie, MSD, Roche, Novartis, Pfizer, Carlomaurizio Montecucco Speakers bureau: AbbVie, Bristol-Myers Squibb, Celgene, Sanofi, Genzyme, Lilly, MSD, Pfizer, UCB, Carlo Salvarani Grant/research support from: Roche, Consultant for: Eli Lilly and Company, Roche, Abbvie, Jean-Emmanuel Kahn: None declared, Bernard Bonnotte: None declared, Cecile-Audrey Durel: None declared, Luc Mouthon: None declared, Xavier Puechal: None declared, Loic Guillevin: None declared, Giacomo Emmi: None declared, Augusto Vaglio: None declared, Benjamin Terrier: None declared

Published
2019

33. Analysis of IL-6 measurement in patients with GCA treated with tocilizumab should consider concomitant treatment with prednisone

Author

Maxime Samson and Bernard Bonnotte

Subjects
0301 basic medicine, medicine.medical_specialty, Immunology, Giant Cell Arteritis, Antibodies, Monoclonal, Humanized, Infections, Gastroenterology, General Biochemistry, Genetics and Molecular Biology, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Tocilizumab, Rheumatology, Prednisone, Recurrence, Internal medicine, medicine, Immunology and Allergy, Humans, Interleukin 6, 030203 arthritis & rheumatology, medicine.diagnostic_test, biology, business.industry, Interleukin-6, Interleukin, medicine.disease, Blockade, Giant cell arteritis, 030104 developmental biology, chemistry, Erythrocyte sedimentation rate, Concomitant, biology.protein, business, medicine.drug
Abstract

We read with great interest the letter published by Berger et al in which the authors showed that relapse-free survival for giant cell arteritis (GCA) treated with tocilizumab (TCZ) was longer in patients with an interleukin (IL) 6 decline (slope ≤0.1).1 In contrast with Berger et al ,1 IL-6 levels at the last TCZ infusion were not found to predict the risk of relapse in our phase 2 prospective open-label clinical trial that included 20 patients with GCA (19/20 patients with new-onset GCA) treated with prednisone and TCZ (8 mg/kg/4 weeks from inclusion to week 12 (W12)). These patients were followed for 52 weeks, and IL-6 levels were monitored by luminex (eBioscience) at inclusion (W0), W12 and W26.2 As a consequence of the blockade of the IL-6 pathway, acute-phase reaction proteins (erythrocyte sedimentation rate, C-reactive protein …

Published
2019

34. Non-severe eosinophilic granulomatosis with polyangiitis: long-term outcomes after remission-induction trial

Author

Achille Aouba, Claire Le Jeunne, Antoine Néel, Bernard Bonnotte, Christian Agard, Vincent Cottin, Gabriel Baron, Pascal Cohen, Nicolas Limal, Marie Wislez, Philippe Ravaud, Christian Pagnoux, Thomas Quemeneur, Benjamin Terrier, Marc Ruivard, Xavier Puéchal, Loïk Geffray, Loïc Guillevin, François Lifermann, Jean-Luc Saraux, and Luc Mouthon

Subjects
Adult, Male, medicine.medical_specialty, Randomization, Exacerbation, Churg-Strauss Syndrome, Severity of Illness Index, Disease-Free Survival, law.invention, Rheumatology, Randomized controlled trial, Interquartile range, law, Recurrence, Internal medicine, Eosinophilic, Azathioprine, medicine, Humans, Pharmacology (medical), Longitudinal Studies, Sinusitis, Glucocorticoids, Asthma, Aged, Randomized Controlled Trials as Topic, Rhinitis, business.industry, Remission Induction, Middle Aged, medicine.disease, Treatment Outcome, Disease Progression, Female, business, Granulomatosis with polyangiitis, Vasculitis, Immunosuppressive Agents
Abstract

Objective In a previous controlled trial, 1-year adjunction of AZA to glucocorticoids (GC) for patients with non-severe, newly diagnosed eosinophilic granulomatosis with polyangiitis (EGPA) failed to lower remission failure, vasculitis relapse and isolated asthma/rhinosinus exacerbation rates, or cumulative GC use at month (M) 24. The aim of this study was to analyse longer-term outcomes to determine whether subsequent vasculitis relapse or isolated asthma/rhinosinus exacerbation (IARE) rates differed. Methods After M24, patients were followed prospectively, being treated based on physicians’ best judgment. Flares and reasons for increased GC dose or immunosuppressant use were recorded, and reviewed according to randomization group to distinguish vasculitis relapses from IAREs according to EGPA Task Force recommendations. Results Fifty EGPA trial participants were followed for a median (interquartile range) of 6.3 (5.4–7.6) years; two (4%) died 11 months post-inclusion. By M24, vasculitis had relapsed in 21/49 (43%) patients and 14/50 (28%) had IAREs. Another patient died 4.8 years post-inclusion (infection). Among nine patients with subsequent vasculitis relapses, three had a major relapse and three had their first relapse after M24; among 25 patients with later IAREs, 17 occurred after M24. At 5 years, respective vasculitis relapse and IARE rates were 48% (95% CI 34.0, 62.6) and 56% (95% CI 41.7, 70.8), with no between-arm differences (P = 0.32 and 0.13). No entry clinical or biological parameter was associated with these outcomes during follow-up. Conclusion These results confirmed that 1-year AZA and GC induction obtained good overall survival but no long-term benefit for non-severe EGPA patients. Vasculitis relapses, occurring mostly during the first 2 years, and IAREs, occurring throughout follow-up, require other preventive treatments. Trial registration ClinicalTrials.gov, https://clinicaltrials.gov, NCT00647166.

Published
2019

35. 354. EFFICACY OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS TREATMENTS ACCORDING TO THE TYPE OF MANIFESTATIONS BASED ON ANALYSIS OF 636 PATIENTS

Author

Giacomo Emmi, Xavier Puéchal, Bernard Bonnotte, Cécile-Audrey Durel, Jean-Emmanuel Kahn, Chiara Marvisi, Maxime Samson, Vítor Teixeira, Matthias Papo, Augusto Vaglio, Matthieu Groh, Sara Monti, Loïc Guillevin, Benjamin Seeliger, Carlo Salvarani, Giulia Cassone, David Jayne, Carlomaurizio Montecucco, M.L. Urban, Franco Schiavon, Renato Alberto Sinico, Benjamin Terrier, Bernhard Hellmich, Luc Mouthon, Juliane Mahrhold, C. Kroegel, Thomas Barba, Thomas Neumann, Papo, M, Sinico, A, Teixeira, V, Urban, M, Mahrhold, J, Cassone, G, Schiavon, F, Groh, M, Marvisi, C, Samson, M, Barba, T, Jayne, D, Hellmich, B, Salvarani, C, Kahn, J, Bonnotte, B, Durel, C, Cohen, P, Puéchal, X, Mouthon, L, Guillevin, L, Emmi, G, Vaglio, A, and Terrier, B

Subjects
medicine.medical_specialty, Cyclophosphamide, business.industry, Omalizumab, medicine.disease, Rheumatology, Prednisone, Internal medicine, medicine, Eosinophilia, Pharmacology (medical), Rituximab, medicine.symptom, Granulomatosis with polyangiitis, business, Vasculitis, EGPA, treatment, corticosteroids, immunosuppressive drugs, Mepolizumab, medicine.drug
Abstract

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg–Strauss) is a small-vessel necrotizing vasculitis characterized by blood and tissue eosinophilia and asthma. Glucocorticoids (GCs) represent the treatment cornerstone. So far, EGPA management has been based on conventional immunosuppressants, but GC-dependence remains frequent. Recently, therapies targeting B cells and interleukin-5 have been prescribed, but data on large cohorts are lacking. Objectives: This study aimed to describe therapeutic management and efficacy of treatments in EGPA patients. Methods: We set up a multicenter European cohort that included 636 EGPA patients. Treatments used, complete remission rates and vasculitis relapse-free survival were recorded. Complete remission was defined as absence of vasculitis relapse and prednisone dose Results: For induction, cyclophosphamide (CYC) was the most frequently prescribed immunosuppressant (36.2%), more often in patients with FFS ≥1 (P Complete remission rates were similar between conventional immunosuppressants (CYC, AZA or MTX) and GCs alone. Vasculitis relapse-free survival was also similar between CYC, AZA or MTX and GCs alone. Similar results were observed for first vasculitis relapse treatments. During follow-up, GC-dependent asthma and/or ENT manifestations were treated with AZA (40%), MTX (25%), mycophenolate mofetil (16%), rituximab (RTX) (21%), CYC (19%), cyclosporine (6%), omalizumab (5.9%) and mepolizumab (5.5%), allowing GC-tapering ≤7.5mg/in 23%, 31%, 17%, 43%, 5%, 71%, 25% and 50%, respectively. Conventional immunosuppressants were mostly used in first and second line, while eosinophil-targeted biotherapies were used in 4th or 5th lines. Conclusion: In EGPA patients, the response to conventional immunosuppressants, in addition to GCs, is often disappointing compared to GCs alone, without clear benefit on complete remission rates and relapse-free survival. In contrast, notwithstanding a small number of treated patients, eosinophil-targeted therapies seemed promising to treat asthma and/or ENT manifestations. Disclosure of Interests: Matthias Papo: None declared, Renato A. Sinico: None declared, Vitor Teixeira: None declared, Maria-Letizia Urban: None declared, Juliane Mahrhold: None declared, Sara Monti: None declared, Giulia Cassone: None declared, Franco Schiavon: None declared, Benjamin Seeliger: None declared, Thomas Neumann: None declared, Claus Kroegel: None declared, Matthieu Groh: None declared, Chiara Marvisi: None declared, Maxime Samson: None declared, Thomas Barba: None declared, David Jayne Grant/research support from: David Jayne has received research grants from Chemocentryx, GSK, Roche/Genentech and Sanofi-Genzyme. He has received consultancy fees from Astra-Zeneca, Boehringer-Ingelheim, Chemocentryx, Chugai, GSK, Infla-RX, Insmed and Takeda, Bernhard Hellmich Consultant for: Roche, Speakers bureau: Abbvie, MSD, Roche, Novartis, Pfizer, Carlomaurizio Montecucco Speakers bureau: AbbVie, Bristol-Myers Squibb, Celgene, Sanofi, Genzyme, Lilly, MSD, Pfizer, UCB, Carlo Salvarani Grant/research support from: Roche, Consultant for: Eli Lilly and Company, Roche, Abbvie, Jean-Emmanuel Kahn: None declared, Bernard Bonnotte: None declared, Cecile-Audrey Durel: None declared, Xavier Puechal: None declared, Luc Mouthon: None declared, Loic Guillevin: None declared, Giacomo Emmi: None declared, Augusto Vaglio: None declared, Benjamin Terrier: None declared

Published
2019

36. 117. Prediction of Long-Term Evolutionary Profiles in Eosinophilic Granulomatosis with Polyangiitis (Churg– Strauss) Based on Baseline and Follow-Up Characteristics

Author

Matthieu Groh, Maria Letizia Urban, Benjamin Terrier, Vítor Teixeira, Augusto Vaglio, Renato Alberto Sinico, Bernard Bonnotte, Bernhard Hellmich, Maxime Samson, Giulia Cassone, Giacomo Emmi, Loïc Guillevin, Xavier Puéchal, Carlo Salvarani, Matthias Papo, Franco Schiavon, David Jayne, Cécile-Audrey Durel, Pascal Cohen, Jean-Emmanuel Kahn, Thomas Barba, Chiara Marvisi, Luc Mouthon, Juliane Mahrhold, Papo, M, Sinico, R, Teixeira, V, Urban, M, Mahrhold, J, Cassone, G, Schiavon, F, Groh, M, Marvisi, C, Samson, M, Barba, T, Jayne, D, Hellmich, B, Salvarani, C, Kahn, J, Bonnotte, B, Durel, C, Cohen, P, Puéchal, X, Mouthon, L, Guillevin, L, Emmi, G, Vaglio, A, and Terrier, B

Subjects
Pediatrics, medicine.medical_specialty, Rheumatology, business.industry, EGPA, outcome, Eosinophilic, medicine, Churg-strauss syndrome, Pharmacology (medical), medicine.disease, Granulomatosis with polyangiitis, business, Churg strauss
Published
2019

37. Antinuclear antibodies in 'infarct like' acute myocarditis: Change in prognosis?

Author

Bernard Bonnotte, Alexandre Cochet, C. Auvens, Maud Maza, Alain Lalande, Marianne Zeller, M. Carré, Yves Cottin, Thibault Leclercq, T. Pommier, Charles Guenancia, and C. Lairet

Subjects
musculoskeletal diseases, Autoimmune disease, medicine.medical_specialty, Acute coronary syndrome, Myocarditis, Anti-nuclear antibody, biology, business.industry, Chest pain, medicine.disease, Troponin, stomatognathic diseases, Acute myocarditis, Internal medicine, cardiovascular system, medicine, biology.protein, Cardiology, cardiovascular diseases, medicine.symptom, skin and connective tissue diseases, Cardiology and Cardiovascular Medicine, business, Blood sampling
Abstract

Background Antinuclear antibodies (ANA) are often positive in patients with “infarct like” acute myocarditis. The objectives of this study were to compare the course of “infarct like” acute myocarditis depending on the presence or absence of ANA at diagnosis. Methods All cases of infarct-like acute myocarditis, revealed as an acute coronary syndrome with chest pain, elevated troponin levels and ECG changes, from 2012 to 2018, were included at Dijon University Hospital. The diagnosis of myocarditis was based on clinical symptoms and was confirmed by cardiac magnetic resonance (CMR). CMR was performed in the acute phase, at 3 months and one year after the acute event. A blood sampling including ANA test is obtained at the moment of the diagnostic. Results Among the patients with “infarct like” acute myocarditis, the ANA assay was performed in 83 patients. In 45 patients (54%), ANA were detected, with a median peak of 160 (n Conclusion Antinuclear antibodies (ANA) are present in patients with “infarct like” acute myocarditis in more than 50% of cases. According to the presence or absence of ANA, the CMR profile is different, both on initial extent and on recovery at distance of the acute phase. These results demonstrate that the presence of ANA, even without other manifestations of an autoimmune disease, influence or affect the course of “infarct like” acute myocarditis.

Published
2021

38. Impact of microbiological samples in the hospital management of community-acquired, nursing home-acquired and hospital-acquired pneumonia in older patients

Author

Bernard Bonnotte, Lionel Piroth, Patrick Manckoundia, Hélène Sordet-Guépet, Philippe d’Athis, Jennifer Tetu, Alain Putot, Henri Bailly, S. Perrin, J.F. Besancenot, P-E Charles, Pascal Chavanet, Service de médecine gériatrique (CHU de Dijon - Centre gériatrique de Champmaillot - EHPAD), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand ( CHU Dijon ), Département d'infectiologie (CHU de Dijon), Service de médecine interne et maladies systémiques (SOC 2) [CHU de Dijon], Service de médecine interne et immunologie clinique (SOC 1) [CHU de Dijon], Service Biostatistiques et Informatique Médicale (CHU de Dijon) ( DIM ), Service de Réanimation Médicale (CHU de Dijon), Cognition, Action, et Plasticité Sensorimotrice [Dijon - U1093] ( CAPS ), Université de Bourgogne ( UB ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Service Biostatistiques et Informatique Médicale (CHU de Dijon) (DIM), Cognition, Action, et Plasticité Sensorimotrice [Dijon - U1093] (CAPS), and Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)

Subjects
Male, diagnosis, severity, Hospital-acquired pneumonia, Severity of Illness Index, 0302 clinical medicine, Medical microbiology, Anti-Infective Agents, Older patients, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, [ SDV.MP ] Life Sciences [q-bio]/Microbiology and Parasitology, adults, Hospital Mortality, guidelines, infections, 030212 general & internal medicine, Aged, 80 and over, validation, Cross Infection, Age Factors, Disease Management, General Medicine, Antimicrobial, Hospitals, 3. Good health, Community-Acquired Infections, Hospitalization, [ SDV.MHEP.MI ] Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Treatment Outcome, [SDV.MP]Life Sciences [q-bio]/Microbiology and Parasitology, Infectious Diseases, Female, Microbiology (medical), medicine.medical_specialty, 03 medical and health sciences, Internal medicine, Intensive care, Severity of illness, medicine, Humans, Aged, Retrospective Studies, business.industry, Retrospective cohort study, Pneumonia, medicine.disease, Nursing Homes, Surgery, 030228 respiratory system, business
Abstract

International audience; We investigated the positivity rate, the detection rates for non-covered pathogens and the therapeutic impact of microbiological samples (MS) in community-acquired pneumonia (CAP), nursing home-acquired pneumonia (NHAP) and hospital-acquired pneumonia (HAP) in elderly hospitalised patients. Patients aged 75 years and over with pneumonia and hospitalised between 1/1/2013 and 30/6/2013 in the departments of medicine (5) and intensive care (1) of our university hospital were included. Microbiological findings, intra-hospital mortality and one-year mortality were recorded. Among the 217 patients included, there were 138 CAP, 56 NHAP and 23 HAP. MS were performed in 89.9, 91.1 and 95.6 % of CAP, NHAP and HAP, respectively. Microbiological diagnosis was made for 29, 11.8 and 27.3 % of patients for CAP, NHAP and HAP, respectively (p = 0.05). Non-covered pathogens were detected for 8 % of CAP, 2 % of NHAP and 13.6 % of HAP (p = 0.1). The antimicrobial spectrum was significantly more frequently reduced when the MS were positive (46.7 % vs. 10.8 % when MS were negative, p = 10(-7)). The MS positivity rate was significantly lower in NHAP than in CAP and HAP. MS revealed non-covered pathogens in only 2 % of NHAP. These results show the poor efficiency and weak clinical impact of MS in the management of pneumonia in hospitalised older patients and suggest that their use should be rationalised.

Published
2016

39. Description of 214 cases of autoimmune congenital heart block: Results of the French neonatal lupus syndrome

Author

Kateri Levesque, Nathalie Morel, Alice Maltret, Gabriel Baron, Agathe Masseau, Pauline Orquevaux, Jean-Charles Piette, Francois Barriere, Jérome Le Bidois, Laurent Fermont, Olivier Fain, Arnaud Theulin, Francois Sassolas, Philippe Pezard, Zahir Amoura, Gaëlle Guettrot-Imbert, Delphine Le Mercier, Sophie Georgin-Lavialle, Christophe Deligny, Eric Hachulla, Luc Mouthon, Philippe Ravaud, Elisabeth Villain, Damien Bonnet, Nathalie Costedoat-Chalumeau, Holy Bezanahary, Boris Bienvenu, Gilles Blaison, Philippe Blanche, Bernard Bonnotte, Pascal Cathebras, Christine Christides, Fleur Cohen, Laurence Cohen, Edouard Devaud, Elisabeth Diot, Pierre Duhaut, Yves Dulac, Bertrand Godeau, Véronique Gournay, Céline Gronier, Loïc Guillevin, Mohamed Hamidou, Julien Haroche, Gilles Hayem, François Heitz, Richard Isnard, Moez Jallouli, Anne-Sophie Korganow, Claire Le Jeunne, François Lhote, Hugues Lucron, Jean-René Lusson, Suzel Magnier, Jacques Ninet, Nicolas Pangaud, Thomas Papo, Jean-Luc Pellegrin, Jean Loup Pennaforte, Jacques Pouchot, Françoise Sarrot-Reynauld, Nicolas Schleinitz, Pascal Seve, Bertrand Stos, Denis Vital-Durand, and Bertrand Wechsler

Subjects
Pacemaker, Artificial, medicine.medical_specialty, Pediatrics, Fetus, Pregnancy, Multivariate analysis, Heart block, business.industry, Immunology, Retrospective cohort study, Dilated cardiomyopathy, medicine.disease, Prosthesis Implantation, Heart Block, Treatment Outcome, In utero, Internal medicine, medicine, Cardiology, Humans, Lupus Erythematosus, Systemic, Immunology and Allergy, Gestation, business
Abstract

Cardiac neonatal lupus syndrome is due to anti-SSA or SSB antibodies and mainly includes congenital heart block (CHB) and dilated cardiomyopathy (DCM). Its optimal management is still debated. We report a large series of autoimmune high degree CHB.Inclusion criteria in this retrospective study were fetuses or neonates with high-degree CHB associated with maternal anti-SSA/SSB antibodies.214 CHB were included: 202 detected in utero at a median term of 23 weeks' gestation (WG) [range 16 to 39 WG] and 12 neonatal cases diagnosed at a median age of 0 days [range birth to 8 days]. The 214 cases of CHB included 202 (94.4%) third-degree CHB, 8 (3.7%) second-degree CHB, and 4 (1.9%) intermittent CHB. In multivariate analysis, the factors associated with feto-neonatal deaths (15.7%) were hydrops (p0.001; hazard ratio [HR] 12.4 [95% confidence interval (95%CI) 4.7-32.7]) and prematurity (p=0.002; HR 17.1 [95%CI 2.8-103.1]). During a median follow-up of 7 years [birth to 36 years], 148 of 187 children born alive (79.1%) had a pacemaker, 35 (18.8%, one missing data) had DCM, and 22 (11.8%) died. In multivariate analysis, factors associated with child death were in utero DCM (p=0.0157; HR 6.37 [95%CI: 1.25-32.44]), postnatal DCM (p0.0001; HR 227.58[95%CI: 24.33-2128.46]) and pacemaker implantation (p=0.0035; HR 0.11[95%CI: 0.02-0.51]). The use of fluorinated steroids was neither associated with survival nor with regression of 2nd degree CHB.In this second largest series of CHB, we confirm some of the previous results. We were unable to find data supporting the routine use of in utero fluorinated steroids.

Published
2015

40. ANCA-associated vasculitides: Recommendations of the French Vasculitis Study Group on the use of immunosuppressants and biotherapies for remission induction and maintenance

Author

Maxime Samson, Yoann Crabol, Bernard Bonnotte, Olivier Aumaître, Noémie Jourde-Chiche, Benjamin Terrier, Mikael Ebbo, Cécile-Audrey Durel, Loïc Guillevin, Alexandre Belot, Xavier Puéchal, Jean-Christophe Lega, Frederic Vandergheynst, Pierre Charles, Thomas Quemeneur, Grégory Pugnet, and Camillo Ribi

Subjects
medicine.medical_specialty, Cardiology, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Maintenance Chemotherapy, 03 medical and health sciences, Remission induction, 0302 clinical medicine, Maintenance therapy, medicine, Humans, 030212 general & internal medicine, Medical prescription, Intensive care medicine, Societies, Medical, 030203 arthritis & rheumatology, business.industry, Remission Induction, Granulomatosis with Polyangiitis, General Medicine, medicine.disease, Biological Therapy, Practice Guidelines as Topic, Rituximab, France, Granulomatosis with polyangiitis, business, Microscopic polyangiitis, Vasculitis, Mepolizumab, Immunosuppressive Agents, medicine.drug
Abstract

Treatment of vasculitides associated with anti-neutrophil cytoplasm antibodies (ANCA) (AAVs) has evolved dramatically in recent years, particularly since the demonstration of rituximab efficacy as remission induction and maintenance therapy for granulomatosis with polyangiitis and microscopic polyangiitis. In 2013, the French Vasculitis Study Group (FVSG) published recommendations for its use by clinicians. Since then, new data have made it possible to better specify and codify prescription of rituximab to treat AAVs. Herein, the FVSG Recommendations Committee, an expert panel comprised of physicians with extensive experience in the treatment and management of vasculitides, presents its consensus guidelines based on literature analysis, the results of prospective therapeutic trials and personal experience.

Published
2020

41. Full-field optical coherence tomography for the diagnosis of giant cell arteritis

Author

Emilie Benoit, Sylvain Audia, Pierre-Henry Gabrielle, Maxime Samson, Hélène Greigert, Olivier Clément, Laurent Martin, Bernard Bonnotte, Jean-Marie Chassot, Daniel Balvay, Claude Boccara, Catherine Creuzot-Garcher, Bertrand Tavitian, Thomas Maldiney, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Université Bourgogne Franche-Comté [COMUE] (UBFC), Institut National de la Santé et de la Recherche Médicale (INSERM), LLTech SAS Paris, Centre des Sciences du Goût et de l'Alimentation [Dijon] (CSGA), Université de Bourgogne (UB)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Centre National de la Recherche Scientifique (CNRS)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Institut Langevin - Ondes et Images (UMR7587) (IL), Sorbonne Université (SU)-Ecole Superieure de Physique et de Chimie Industrielles de la Ville de Paris (ESPCI Paris), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université de Paris (UP)-Centre National de la Recherche Scientifique (CNRS), Paris-Centre de Recherche Cardiovasculaire (PARCC (UMR_S 970/ U970)), 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)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), Julien, Sabine, Ecole Superieure de Physique et de Chimie Industrielles de la Ville de Paris (ESPCI Paris), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), and 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)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité)

Subjects
Male, genetic structures, Biopsy, Pathology and Laboratory Medicine, 01 natural sciences, Diagnostic Radiology, Cohort Studies, 0302 clinical medicine, Medicine and Health Sciences, Prospective Studies, skin and connective tissue diseases, Tomography, Immune Response, Aged, 80 and over, Microscopy, Multidisciplinary, medicine.diagnostic_test, Radiology and Imaging, Light Microscopy, Software Engineering, Arteries, Temporal artery biopsy, Middle Aged, Temporal Arteries, medicine.anatomical_structure, [SDV.MHEP.OS] Life Sciences [q-bio]/Human health and pathology/Sensory Organs, cardiovascular system, Medicine, Engineering and Technology, Female, Temporal artery, Radiology, Anatomy, Tunica Media, Tomography, Optical Coherence, Research Article, Computer and Information Sciences, medicine.medical_specialty, Histology, Imaging Techniques, Science, Immunology, Giant Cell Arteritis, Surgical and Invasive Medical Procedures, Research and Analysis Methods, Computer Software, 010309 optics, Interference Microscopy, 03 medical and health sciences, Signs and Symptoms, Optical coherence tomography, Diagnostic Medicine, 0103 physical sciences, medicine, Humans, [SDV.MHEP.OS]Life Sciences [q-bio]/Human health and pathology/Sensory Organs, Aged, Inflammation, 030203 arthritis & rheumatology, business.industry, Biology and Life Sciences, Full field, medicine.disease, Internal elastic lamina, Giant cell arteritis, Anatomical Pathology, Surgical Pathology, Case-Control Studies, Vasa vasorum, Reticular connective tissue, Cardiovascular Anatomy, Blood Vessels, Clinical Medicine, Tunica Intima, business
Abstract

Histopathological examination of temporal artery biopsy (TAB) remains the gold standard for the diagnosis of giant cell arteritis (GCA) but is associated with essential limitations that emphasize the need for an upgraded pathological process. This study pioneered the use of full-field optical coherence tomography (FF-OCT) for rapid and automated on-site pathological diagnosis of GCA. Sixteen TABs (12 negative and 4 positive for GCA) were selected according to major histopathological criteria of GCA following hematoxylin-eosin-saffron-staining for subsequent acquisition with FF-OCT to compare structural modifications of the artery cell wall and thickness of each tunica. Gabor filtering of FF-OCT images was then used to compute TAB orientation maps and validate a potential automated analysis of TAB sections. FF-OCT allowed both qualitative and quantitative visualization of the main structures of the temporal artery wall, from the internal elastic lamina to the vasa vasorum and red blood cells, unveiling a significant correlation with conventional histology. FF-OCT imaging of GCA TABs revealed destruction of the media with distinct remodeling of the whole arterial wall into a denser reticular fibrous neo-intima, which is distinctive of GCA pathogenesis and accessible through automated Gabor filtering. Rapid on-site FF-OCT TAB acquisition makes it possible to identify some characteristic pathological lesions of GCA within a few minutes, paving the way for potential machine intelligence-based or even non-invasive diagnosis of GCA.

Published
2020

42. Systemic lupus erythematosus and neutropaenia: a hallmark of haematological manifestations

Author

Aurore, Meyer, Aurélien, Guffroy, Gilles, Blaison, Yannick, Dieudonne, Zahir, Amoura, Bernard, Bonnotte, Christoph, Fiehn, Pierre, Kieffer, Hannes Martin, Lorenz, Nadine, Magy-Bertrand, François, Maurier, Jean-Louis, Pennaforte, Hans-Hartmut, Peter, Andreas, Schwarting, Jean, Sibilia, Laurent, Arnaud, Thierry, Martin, Reinhard Edmund, Voll, Anne-Sophie, Korganow, R, Voll, CHU Strasbourg, Université de Strasbourg (UNISTRA), Immuno-Rhumatologie Moléculaire, Université de Strasbourg (UNISTRA)-Institut National de la Santé et de la Recherche Médicale (INSERM), Fédération Hospitalo-Universitaire OMICARE, Fédération de Médecine Translationnelle de Strasbourg (FMTS), Médecine interne [Hôpitaux civils de Colmar], Hôpitaux Civils de Colmar, Institut E3M [CHU Pitié-Salpêtrière], 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), CHU Dijon, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre Hospitalier Emile Muller [Mulhouse] (CH E.Muller Mulhouse), Groupe Hospitalier de Territoire Haute Alsace (GHTHA), Heidelberg University Hospital [Heidelberg], Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Hôpitaux Privés de Metz (HPMetz), Centre Hospitalier Universitaire de Reims (CHU Reims), University of Freiburg [Freiburg], Les Hôpitaux Universitaires de Strasbourg (HUS), and LBBR/Rarenet group: Z Amoura, L Arnaud, G Blaison, B Bonnotte, E Chatelus, E Ciobanu, F Duchene, J P Faller, A Gorse, J E Gottenberg, O Hinschberger, F Jaeger, P Kieffer, M Kilifa, N Magy-Bertrand, T Martin, L Martzolff, F Maurier, A Meyer, J-L Pasquali, J-L Pennaforte, V Poindron, S Revuz, M Samson, J Sibilia, C Sordet, A Theulin, D Wahl, J C Weber, M Bartsch, N Bartholomä, C Fiehn, S Finzel, A Funkert, M Hausberg, H Lorenz, R Max, H-H Peter, M Rizzi, A Schwarting, J Thiel, N Venhoff, R Voll

Subjects
Adult, Male, 0301 basic medicine, medicine.medical_specialty, Neutropenia, autoantibodies, Immunology, Disease, Sciences du Vivant [q-bio]/Médecine humaine et pathologie, Autoimmune Diseases, 03 medical and health sciences, 0302 clinical medicine, Germany, Lymphopenia, lupus erythematosus, systemic, Internal medicine, medicine, Humans, Oral ulcers, 030203 arthritis & rheumatology, Autoimmune disease, Systemic lupus erythematosus, Genetic heterogeneity, business.industry, Autoantibody, Complement C3, General Medicine, systemic, medicine.disease, Thrombocytopenia, Epidemiology and Outcomes, 3. Good health, Cross-Sectional Studies, Sjogren's Syndrome, 030104 developmental biology, Antibodies, Antinuclear, Case-Control Studies, Cohort, Absolute neutrophil count, Female, France, business, lupus erythematosus, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract

ObjectiveSystemic lupus is a chronic autoimmune disease characterised by its phenotypic heterogeneity. Neutropaenia is a frequent event in SLE occurring in 20%–40% of patients depending on the threshold value of neutrophil count. On a daily basis, the management of neutropaenia in SLE is difficult with several possible causes. Moreover, the infectious consequences of neutropaenia in SLE remain not well defined.Methods998 patients from the Lupus BioBank of the upper Rhein (LBBR), a large German and French cohort of patients with SLE, mostly of Caucasian origin (83%), were included in this study. Neutropaenia was considered when neutrophil count was below 1800×106/L. An additional analysis of detailed medical records was done for 65 LBBR patients with neutropaenia.Results208 patients with neutropaenia (21%) were compared with 779 SLE patients without neutropaenia. Neutropaenia in SLE was significantly associated with thrombocytopaenia (OR 4.11 (2.57–10.3)), lymphopaenia (OR 4.41 (2.51–11.5)) and low C3 (OR 1.91 (1.03–4.37)) in multivariate analysis. 65 representative patients with neutropaenia were analysed. Neutropaenia was moderate to severe in 38%, chronic in 31%, and both severe and chronic in 23% of cases. Moderate to severe and chronic neutropaenia were both associated with lymphopaenia and thrombopaenia. Chronic neutropaenia was also associated anti-Ro/SSA antibodies and moderate to severe neutropaenia with oral ulcers.ConclusionThis study is to date the largest cohort to describe neutropaenia in SLE. Neutropaenia displays a strong association with other cytopaenias, suggesting a common mechanism. Chronic neutropaenia is associated with anti-Ro/SSA antibodies with or without identified Sjögren’s disease.

Published
2020

43. Venous thromboembolic events during warm autoimmune hemolytic anemia

Author

Bernard Bonnotte, Laurence Duvillard, Maxime Samson, Benoit Bach, Daniela Lakomy, Marine Branger, Sabine Berthier, Sylvain Audia, Jean Baptiste Bour, Marc Michel, Bénédicte Burlet, Julien Guy, Vanessa Leguy-Seguin, Service de médecine interne et immunologie clinique (SOC 1) [CHU de Dijon], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre de référence des maladies rares des cytopénies auto-immunes de l'adulte (CHU Dijon) (CRMR des cytopénies auto-immunes de l'adulte ), Laboratoire d'Immunologie et d'Immunothérapie des cancers [Dijon] (LIIC), École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université de Bourgogne (UB), Laboratoire de sérologie-virologie (CHU de Dijon), Service d'hématologie biologique (CHU de Dijon), Service de Biochimie [CHU de Dijon], 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), and Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université de Bourgogne (UB)-Université Bourgogne Franche-Comté [COMUE] (UBFC)

Subjects
Hemolytic anemia, Male, Epidemiology, medicine.medical_treatment, lcsh:Medicine, Disease, 030204 cardiovascular system & hematology, Cardiovascular Medicine, Vascular Medicine, Biochemistry, Leukocyte Count, 0302 clinical medicine, Risk Factors, Medicine and Health Sciences, lcsh:Science, Multidisciplinary, Anemia, Venous Thromboembolism, Hematology, Middle Aged, Thrombosis, 3. Good health, Deep Vein Thrombosis, Oncology, Cardiovascular Diseases, Female, Autoimmune hemolytic anemia, Research Article, Adult, medicine.medical_specialty, Splenectomy, 03 medical and health sciences, Thrombotic manifestations, Diagnostic Medicine, Internal medicine, Thromboembolism, medicine, Cancer Detection and Diagnosis, Humans, Hemoglobin, cardiovascular diseases, Blood Coagulation, Aged, Retrospective Studies, Coagulation Disorders, business.industry, lcsh:R, Biology and Life Sciences, Proteins, Anticoagulants, Retrospective cohort study, Bilirubin, Odds ratio, medicine.disease, equipment and supplies, Auto-immune diseases (AID), Medical Risk Factors, Multivariate Analysis, lcsh:Q, Anemia, Hemolytic, Autoimmune, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, 030215 immunology
Abstract

International audience; Thrombotic manifestations are a hallmark of many auto-immune diseases (AID), specially of warm autoimmune hemolytic anemia (wAIHA), as 15 to 33% of adults with wAIHA experience venous thromboembolic events (VTE). However, beyond the presence of positive antiphospholipid antibodies and splenectomy, risk factors for developing a VTE during wAIHA have not been clearly identified. The aim of this retrospective study was to characterize VTEs during wAIHA and to identify risk factors for VTE. Forty-eight patients with wAIHA were included, among whom 26 (54%) had secondary wAIHA. Eleven (23%) patients presented at least one VTE, that occurred during an active phase of the disease for 10/11 patients (90%). The frequency of VTE was not different between primary and secondary AIHA (23.7 vs. 19.2%; p = 0.5). The Padua prediction score based on traditional risk factors was not different between patients with and without VTE. On multivariate analysis, total bilirubin ≥ 40 μmol/L [odds ratio (OR) = 7.4; p = 0.02] and leucocyte count above 7x109/L (OR = 15.7; p = 0.02) were significantly associated with a higher risk of thrombosis. Antiphospholipid antibodies were screened in 9 out the 11 patients who presented a VTE and were negative. Thus, the frequency of VTE is high (23%) during wAIHA and VTE preferentially occur within the first weeks of diagnosis. As no clinically relevant predictive factors of VTE could be identified, the systematic use of a prophylactic anticoagulation should be recommended in case of active hemolysis and its maintenance after hospital discharge should be considered. The benefit of a systematic screening for VTE and its procedure remain to be determined.

Published
2018

44. Predictors of fatigue and severe fatigue in a large international cohort of patients with systemic lupus erythematosus and a systematic review of the literature

Author

Rheinardt Voll, Hannes M. Lorenz, Gilles Blaison, Pierre Kieffer, Bernard Bonnotte, Laurent Arnaud, Zahir Amoura, Vincent Poindron, Jean-Loup Pennaforte, Jean Sibilia, Thierry Martin, Pierre Edouard Gavand, Nadine Magy-Bertrand, Hans-Harmut Peter, François Maurier, Andreas Schwarting, and Christoph Fiehn

Subjects
Adult, Male, medicine.medical_specialty, Anxiety, Logistic regression, Severity of Illness Index, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Rheumatology, Interquartile range, Risk Factors, Internal medicine, medicine, Humans, Lupus Erythematosus, Systemic, Pharmacology (medical), 030212 general & internal medicine, Depression (differential diagnoses), Fatigue, Aged, 030203 arthritis & rheumatology, Systemic lupus erythematosus, Lupus erythematosus, business.industry, Depression, Odds ratio, Middle Aged, medicine.disease, Cohort, Quality of Life, Female, medicine.symptom, business
Abstract

OBJECTIVE Fatigue is reported in up to 90% of patients with SLE. This study was conducted to identify the determinants associated with fatigue in a large cohort of patients with SLE, as well as to provide a systematic review of the literature. METHODS Patients from the Lupus BioBank of the upper Rhein, a large German-French cohort of SLE patients, were included in the FATILUP study if they fulfilled the 1997 ACR criteria for SLE and had Fatigue Scale for Motor and Cognitive Functions scores collected. Multivariate logistic regression analyses were performed to assess the determinants of fatigue and severe fatigue. RESULTS A total of 570 patients were included (89.1% female). The median age was 42 years (interquartile range 25-75: 34-52). The median value of the SAfety of Estrogens in Lupus Erythematosus National Assessment (SELENA)-SLEDAI was 2 (0-4). Fatigue was reported by 386 patients (67.7%) and severe fatigue by 209 (36.7%). In multivariate analyses, fatigue was associated with depression [odds ratio (OR): 4.72 (95% CI: 1.39-16.05), P = 0.01], anxiety [OR: 4.49 (95% CI: 2.60-7.77), P < 0.0001], glucocorticoid treatment [OR: 1.59 (95% CI 1.05-2.41), P = 0.04], SELENA-SLEDAI scores [OR: 1.05 (95% CI: 1.00-1.12) per 1 point increase, P = 0.043] and age at sampling [OR: 1.01 (95% CI: 1.00-1.03) per 1 year increase, P = 0.03]. Severe fatigue was independently associated with anxiety (P < 0.0001), depression (P < 0.0001), glucocorticoid treatment (P = 0.047) and age at sampling (P = 0.03). CONCLUSION Both fatigue and severe fatigue are common symptoms in SLE, and are strongly associated with depression and anxiety. Disease activity and the use of glucocorticoids were also independently associated with fatigue, although more weakly.

Published
2018

45. SAT0459 Occurrence of lymphoma in systemic lupus erythematosus: a case-series of 38 patients

Author

M. Roriz, Christian Lavigne, S. Choquet, Christophe Deligny, Frédéric Charlotte, J.-S. Allain, A. Mathian, Véronique Leblond, Tessa Huscenot, Z. Amoura, J. Haroche, Bernard Bonnotte, N. Costedoat-Chalumeau, Micheline Pha, Grégory Pugnet, Maxime Samson, A.S. Korganow, Thierry Martin, Mathilde Versini, Hervé Devilliers, S. Mouly, V. Le Guern, M. Martin, M. Hie, Fleur Cohen-Aubart, and Pierre-Yves Jeandel

Subjects
Series (stratigraphy), medicine.medical_specialty, business.industry, medicine, medicine.disease, business, Dermatology, Lymphoma
Published
2018

46. Large-vessel involvement and aortic dilation in giant-cell arteritis. A multicenter study of 549 patients

Author

Maxime Samson, Eric Liozon, Aurélie Daumas, David Saadoun, Mikael Ebbo, Bernard Bonnotte, Hubert de Boysson, A. Dumont, Boris Bienvenu, Alain Manrique, Marc Lambert, Audrey Sultan, Achille Aouba, Jean-Jacques Parienti, Mathieu Vautier, 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), Aix Marseille Université (AMU), 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), Unité de Biostatistique et de Recherche Clinique (UBRC), Service de Médecine interne A et polyclinique médicale [CHU Limoges], CHU Limoges, Service de médecine interne [Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Médecine Interne [Hôpital Saint-Joseph - Marseille], Aix Marseille Université (AMU)-Hôpital Saint-Joseph [Marseille], Service de médecine interne et immunologie clinique (SOC 1) [CHU de Dijon], Service de médecine nucléaire [CHU Caen], Institut National de la Santé et de la Recherche Médicale (INSERM), Centre National de la Recherche Scientifique (CNRS), CHU Pitié-Salpêtrière [AP-HP], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)

Subjects
Male, medicine.medical_specialty, Giant Cell Arteritis, Immunology, Aortic Diseases, Large vessel, Disease, 030204 cardiovascular system & hematology, 03 medical and health sciences, 0302 clinical medicine, immune system diseases, Internal medicine, medicine, Humans, Immunology and Allergy, In patient, cardiovascular diseases, Arteritis, skin and connective tissue diseases, Aortic dilation, Aged, Retrospective Studies, 030203 arthritis & rheumatology, Giant-cell arteritis, business.industry, Aortitis Large-vessel involvement, Middle Aged, medicine.disease, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, Giant cell arteritis, Increased risk, [SDV.MP]Life Sciences [q-bio]/Microbiology and Parasitology, Multicenter study, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, Cardiology, cardiovascular system, Female, business
Abstract

International audience; ObjectivesLarge-vessel involvement (LVI) can occur in giant-cell arteritis (GCA) and may represent a distinct disease subgroup with a higher risk for aortic dilation. This study aimed to better characterize the presentation and evolution of LVI in patients with GCA.Patients and methodsA retrospective multicenter study enrolled 248 GCA patients with LVI and 301 GCA patients without LVI on imaging. Factors associated with aortic dilation were identified in a multivariable model.ResultsThe patients with LVI were younger (p < 0.0001), more likely to be women (p = 0.01), and showed fewer cephalic symptoms (p < 0.0001) and polymyalgia rheumatica (p = 0.001) but more extracranial vascular symptoms (p = 0.05) than the patients without LVI. Glucocorticoids (GC) management did not differ between the two groups, but the GC discontinuation rate was lower in the patients with LVI (p = 0.0003). Repeated aortic imaging procedures were performed at 19 months [range: 5–162 months] and 17 months [range: 6–168 months] after diagnosis in 154 patients with LVI and 123 patients without LVI, respectively, of whom 21% and 7%, respectively, presented new aortic dilations (p = 0.0008). In the patients with LVI, aortic dilation occurred on an aorta segment shown to be inflammatory on previous imaging in 94% of patients. In the multivariate analysis, LVI was the strongest predictor of aortic dilation (hazard ratio: 3.16 [range: 1.34–7.48], p = 0.009).ConclusionsLVI represents a distinct disease pattern of GCA with an increased risk of aortic dilation. Control of the aortic morphology during follow-up is required.

Published
2018

47. Tocilizumab as an add-on therapy to glucocorticoids during the first 3 months of treatment of Giant cell arteritis: A prospective study

Author

Jean-François Besancenot, Sylvain Audia, Boris Bienvenu, Kim Heang Ly, Xavier Mariette, Benjamin Terrier, Christine Binquet, Romaric Loffroy, Eric Liozon, Anne Laure Fauchais, Bernard Bonnotte, Raphaèle Seror, Pierre Charles, Hervé Devilliers, Loïc Guillevin, Maxime Samson, François Maurier, Service de médecine interne et immunologie clinique (SOC 1) [CHU de Dijon], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Interactions hôte-greffon-tumeur, ingénierie cellulaire et génique - UFC (UMR INSERM 1098) (HOTE GREFFON), Institut National de la Santé et de la Recherche Médicale (INSERM)-Etablissement français du sang [Bourgogne-France-Comté] (EFS [Bourgogne-France-Comté])-Université de Franche-Comté (UFC), Service de Médecine Interne (SOC 1 et SOC 2) [CHU de Dijon], Centre d'Investigation Clinique 1432 (Dijon) - Epidemiologie Clinique/Essais Cliniques (CIC-EC), Université de Bourgogne (UB)-Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Médecine interne A et polyclinique médicale [CHU Limoges], CHU Limoges, Service de médecine interne, Centre hospitalier régional Metz-Thionville (CHR Metz-Thionville)-Hôpital Sainte-Blandine, Service de médecine interne (Hôpital Belle Isle, Metz), CH Belle-Isle, 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), Institut Mutualiste de Montsouris (IMM), Université Paris Descartes - Paris 5 (UPD5), Service de radiologie et d'Imagerie médicale diagnostique et thérapeutique (CHU de Dijon), Immunologie des Maladies Virales et Autoimmunes (IMVA - U1184), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Sud - Paris 11 (UP11)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA), Interactions hôte-greffon-tumeur, ingénierie cellulaire et génique - UFC (UMR INSERM 1098) (RIGHT), 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]), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université Paris-Sud - Paris 11 (UP11)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA), 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 Sainte-Blandine-Centre hospitalier régional Metz-Thionville (CHR Metz-Thionville), and Université Paris-Sud - Paris 11 (UP11)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Institut National de la Santé et de la Recherche Médicale (INSERM)

Subjects
0301 basic medicine, Male, medicine.medical_specialty, Giant Cell Arteritis, Antibodies, Monoclonal, Humanized, Proof of Concept Study, Drug Administration Schedule, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Tocilizumab, Prednisone, Recurrence, Internal medicine, Internal Medicine, medicine, Clinical endpoint, Humans, Prospective Studies, Relapse, Prospective cohort study, Adverse effect, Glucocorticoids, Aortitis, Aged, 030203 arthritis & rheumatology, business.industry, Interleukin-6, Remission Induction, medicine.disease, 3. Good health, Discontinuation, Temporal Arteries, Clinical trial, Giant cell arteritis, 030104 developmental biology, C-Reactive Protein, Treatment Outcome, chemistry, Positron-Emission Tomography, Female, France, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, medicine.drug
Abstract

Background The aim of this study was to evaluate tocilizumab (TCZ) as an add-on therapy to glucocorticoids (GC) during the first 3 months of treatment of giant cell arteritis (GCA). Methods GCA patients, as defined by ≥3/5 ACR criteria and positive temporal artery biopsy (TAB) or angio-CT-scan or PET-scan-proven aortitis, were included in this prospective open-label study. Prednisone was started at 0.7 mg/kg/day and then tapered according to a standardized protocol. All patients received four infusions of TCZ (8 mg/kg/4 weeks) after inclusion. The primary endpoint was the percentage of patients in remission with ≤0.1 mg/kg/day of prednisone at week 26 (W26). Patients were followed for 52 weeks and data prospectively recorded. Results Twenty patients with a median (IQR) age of 72 (69–78) years were included. TAB were positive in 17/19 (90%) patients and 7/16 (44%) had aortitis. Remission was obtained in all cases. At W26, 15 (75%) patients met the primary endpoint. Ten patients experienced relapse during follow-up, mainly patients with aortitis (P = 0.048), or CRP >70 mg/L (P = 0.036) or hemoglobin ≤10 g/dL (P = 0.015) at diagnosis. Among 64 adverse events (AE) reported in 18 patients, three were severe and 30, mostly non-severe infections (n = 15) and hypercholesterolemia (n = 8), were imputable to the study. Conclusion This study shows that an alternative strategy using a short-term treatment with TCZ can be proposed to spare GC for the treatment of GCA. However, 50% of patients experienced relapse during the 9 months following TCZ discontinuation, especially patients with aortitis, or CRP > 70 mg/L or Hb ≤ 10 g/dL at diagnosis. Trial registration: ClinicalTrials.gov (NCT01910038).

Published
2017

48. Dépistage et prise en charge du risque cardiovasculaire au cours du lupus systémique : élaboration de recommandations pour la pratique clinique, à partir d’une analyse de la littérature et de l’avis d’experts

Author

Philippe Remy, V. Baudouin, M. Chauchard, T. Papo, A. Ghali, Laurent Arnaud, A.S. Korganow, P. Gobert, O. Meyer, A. Viau Brabant, P. Marianetti-Guingel, Viviane Queyrel, N. Martin Silva, Aurélie Hummel, T. Kwon, N. Magy-Bertrand, A. Le Quellec, Noémie Jourde-Chiche, Jean-Loup Pennaforte, V. Le Guern, Thierry Martin, Bernard Bonnotte, Sandrine Morell-Dubois, Jean-François Viallard, M. Hamidou, Nicolas Limal, Makoto Miyara, E. Hachulla, Eric Daugas, A. Chaib, Cristina Belizna, F. Boumedine, Zahir Amoura, Julien Haroche, Denis Wahl, I. Raymond, Brigitte Bader-Meunier, J. Sibilia, Karim Sacre, Guillaume Gondran, E. Bruckert, Laurent Chiche, Jeannot Schmidt, K. Polomat, Baptiste Hervier, Jacques Ninet, Daniel Adoue, Jacques Pourrat, Alexis Mathian, and Gaëlle Guettrot-Imbert

Subjects
Gynecology, medicine.medical_specialty, business.industry, Cardiovascular risk factors, Gastroenterology, Internal Medicine, medicine, business
Abstract

Resume Propos L’objectif de ce travail etait de proposer des recommandations relatives aux modalites de depistage et de prise en charge des facteurs de risque cardiovasculaire au cours du lupus systemique. Methodes Trente-neuf experts internistes, rhumatologues ou nephrologues francais, membres du reseau des centres de reference et de competence du lupus systemique ont pris part a un vote visant a retenir ou non des recommandations preparees par un groupe de travail a partir d’une revue de la litterature. Resultats Les experts ont souligne que les facteurs de risque cardiovasculaires modifiables doivent etre recherches au moins annuellement au cours du lupus systemique. La prescription d’une statine en prevention primaire depend du taux de LDL-cholesterol et du nombre de facteurs de risque cardiovasculaires, en considerant le lupus systemique comme un facteur de risque cardiovasculaire supplementaire. En prevention secondaire, les experts s’accordent a proposer une cible de LDL-cholesterol Conclusions Ces recommandations vont permettre d’ameliorer et d’homogeneiser la pratique des differents medecins francais impliques dans la prise en charge du lupus systemique. L’actualisation de ces recommandations pourra etre ulterieurement envisagee, en fonction des nouvelles donnees publiees.

Published
2015

49. Long-term efficacy of remission-maintenance regimens for ANCA-associated vasculitides

Author

Olivier Aumaître, Olivier Lidove, Chahéra Khouatra, Olivier Decaux, Pascal Cohen, P. Gobert, Claire Blanchard-Delaunay, Philippe Ravaud, Pascal Godmer, Xavier Puéchal, Mohamed Hamidou, Hélène Desmurs-Clavel, Pierre-Louis Carron, Elodie Perrodeau, Marize Ducret, Bernard Bonnotte, Thomas Quemeneur, Christian Pagnoux, François Maurier, Benjamin Terrier, Eric Daugas, Luc Mouthon, Nicolas Limal, Loïc Guillevin, A. Karras, 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), Modèles et méthodes de l'évaluation thérapeutique des maladies chroniques, Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Clermont-Ferrand, Institut de Génétique et Développement de Rennes (IGDR), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Centre National de la Recherche Scientifique (CNRS)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Hôpital Edouard Herriot [CHU - HCL], Hospices Civils de Lyon (HCL), CHU Valenciennes, Santé - STIC, Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Grenoble, Immunopathologie rénale, récepteurs et inflammation, Centre hospitalier universitaire de Nantes (CHU Nantes), Université Paris Descartes - Paris 5 (UPD5), Institut Cochin ( 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 Diderot - Paris 7 ( UPD7 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Institut de Génétique et Développement de Rennes ( IGDR ), Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Centre National de la Recherche Scientifique ( CNRS ) -Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Hospices Civils de Lyon ( HCL ), Université de Bourgogne ( UB ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Centre hospitalier universitaire de Nantes ( CHU Nantes ), Université Paris Descartes - Paris 5 ( UPD5 ), Modèles et méthodes de l'évaluation thérapeutique des maladies chroniques (U738 / UMR_S738), Université de Rennes (UR)-Centre National de la Recherche Scientifique (CNRS)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Centre National de la Recherche Scientifique (CNRS)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Diderot - Paris 7 (UPD7), Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Centre National de la Recherche Scientifique (CNRS)-Université de Rennes 1 (UR1), and Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)

Subjects
Male, Azathioprine, Kaplan-Meier Estimate, urologic and male genital diseases, Gastroenterology, Severity of Illness Index, 0302 clinical medicine, immune system diseases, Recurrence, Risk Factors, Clinical endpoint, Immunology and Allergy, 030212 general & internal medicine, skin and connective tissue diseases, treatment, Remission Induction, Middle Aged, 3. Good health, Treatment Outcome, Antirheumatic Agents, Rituximab, Female, systemic vasculitis, Granulomatosis with polyangiitis, Microscopic polyangiitis, Vasculitis, Immunosuppressive Agents, medicine.drug, Systemic vasculitis, Adult, medicine.medical_specialty, Immunology, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, General Biochemistry, Genetics and Molecular Biology, Drug Administration Schedule, Antibodies, Antineutrophil Cytoplasmic, 03 medical and health sciences, Rheumatology, Internal medicine, medicine, Humans, cardiovascular diseases, Lymphocyte Count, Glucocorticoids, Anti-neutrophil cytoplasmic antibody, Aged, 030203 arthritis & rheumatology, [SDV.GEN]Life Sciences [q-bio]/Genetics, granulomatosis with polyangiitis, Dose-Response Relationship, Drug, business.industry, medicine.disease, respiratory tract diseases, [ SDV.GEN ] Life Sciences [q-bio]/Genetics, business
Abstract

International audience; Objective - To compare long-term efficacy of remission-maintenance regimens in patients with newly diagnosed or relapsing antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides. Methods - The 28-month Maintenance of Remission using Rituximab in Systemic ANCA-associated Vasculitis trial compared rituximab with azathioprine to maintain remission in patients with newly diagnosed or relapsing granulomatosis with polyangiitis, microscopic polyangiitis or renal-limited ANCA-associated vasculitis. Thereafter, prospective patient follow-up lasted until month 60. The primary endpoint was the major-relapse rate at month 60. Relapse and serious adverse event-free survival were also assessed. Results - Among the 115 enrolled patients, only one was lost to follow-up at month 60. For the azathioprine and rituximab groups, respectively, at month 60, the major relapse-free survival rates were 49.4% (95% CI 38.0% to 64.3%) and 71.9% (95% CI 61.2% to 84.6%) (p=0.003); minor and major relapse-free survival rates were 37.2% (95% CI 26.5% to 52.2%) and 57.9% (95% CI 46.4% to 72.2%) (p=0.012); overall survival rates were 93.0% (95% CI 86.7% to 99.9%) and 100% (p=0.045) and cumulative glucocorticoid use was comparable. Quality-adjusted time without symptoms and toxicity analysis showed that rituximab-treated patients had 12.6 months more without relapse or toxicity than those given azathioprine (p

Published
2017

50. Efficacy and safety of dapsone as second line therapy for adult immune thrombocytopenia: A retrospective study of 42 patients

Author

Sabine Berthier, Maxime Samson, Bernard Bonnotte, Barbara Nicolas, Clémentine Estève, Sylvain Audia, Vanessa Leguy-Seguin, and Alexandre Guilhem

Subjects
Adult, medicine.medical_specialty, medicine.medical_treatment, Splenectomy, lcsh:Medicine, 030204 cardiovascular system & hematology, Dapsone, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, Humans, Adverse effect, lcsh:Science, Retrospective Studies, Second-line therapy, Purpura, Thrombocytopenic, Idiopathic, Multidisciplinary, business.industry, lcsh:R, Retrospective cohort study, Immune thrombocytopenia, Surgery, Sustained response, Cohort, lcsh:Q, business, 030215 immunology, medicine.drug, Research Article
Abstract

Dapsone is recommended as a second line therapy in immune thrombocytopenia (ITP), but is underused because of its potential side effects. The medical charts of 42 ITP patients treated with dapsone (100 mg/day) were retrospectively reviewed in order to assess its efficacy and safety in daily clinical practice. The overall response rate was 54.8% (n = 22, with a complete response in 38.1%) with a median time to response of 29 days (24-41 days). Patients with complete response had shorter disease duration whereas no difference was observed between responders and non-responders regarding age, sex or previous treatments received. Importantly, after dapsone withdrawal, a sustained response was observed in 5 patients, representing 12% of the whole cohort. Twenty percent of patients (n = 8) relapsed on therapy after 8.1 (6.5-13.6) months. Side effects occurred in 31% (n = 13) of patients, and required dapsone withdrawal in 22% (n = 9) or dosage reduction in 10% (n = 4) of the cases. Side effects resolved in all but one case. Overall, these data support dapsone as an interesting second line therapy in ITP, with a good safety and efficacy profile at a low cost.

Published
2017

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