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1. Antiplatelet and anticoagulant therapies in hereditary hemorrhagic telangiectasia: A large French cohort study (RETROPLACOTEL)

Author

Grobost, Vincent, Hammi, Sami, Pereira, Bruno, Guilhem, Alexandre, Duffau, Pierre, Seguier, Julie, Parrot, Antoine, Gautier, Giovanni, Alric, Laurent, Kerjouan, Mallorie, Le Guillou, Xavier, Simon, Delphine, Chaussavoine, Laurent, Rondeau-Lutz, Murielle, Leguy-Seguin, Vanessa, Delagrange, Laura, Lavigne, Christian, Maillard, Hélène, and Dupuis-Girod, Sophie

Published
2023
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2. Development and validation of a quality of life measurement scale specific to hereditary hemorrhagic telangiectasia: the QoL-HHT

Author

Le, Thi Thao Truc, Martinent, Guillaume, Dupuis-Girod, Sophie, Parrot, Antoine, Contis, Anne, Riviere, Sophie, Chinet, Thierry, Grobost, Vincent, Espitia, Olivier, Dussardier-Gilbert, Brigitte, Alric, Laurent, Armengol, Guillaume, Maillard, Hélène, Leguy-Seguin, Vanessa, Leroy, Sylvie, Rondeau-Lutz, Murielle, Lavigne, Christian, Mohamed, Shirine, Chaussavoine, Laurent, Magro, Pascal, Seguier, Julie, Kerjouan, Mallorie, and Fourdrinoy, Sylvie

Published
2022
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4. COVID-19 outcomes in patients with inflammatory rheumatic and musculoskeletal diseases treated with rituximab: a cohort study

Author

Aeschlimann, Florence, Agard, Christian, Ait-Abdallah, Nassim, Albert, Jean-David, Alcais, Didier, Allain, Jean-Sébastien, Allanore, Yannick, Amador-Borreiro, Blanca, Amoura, Zahir, Andre, Emma, Arbault, Anaïs, Arlet, Jean-Benoît, Arnaud, Laurent, Arniaud, Denis, Arty-Hue, Herliette, Atlan, Lucie, Audemard-Verger, Alexandra, Audoin-Pajot, Christine, Audren, Victor, Bach-Bunner, Maxime, Bacquet-Deschryver, Hélène, Bader-Meunier, Brigitte, Balandraud, Nathalie, Balblanc, Jean-Charles, Bally, Stéphane, Banal, Frédéric, Barbery, Pierre, Barnetche, Thomas, Barrelet, Audre, Basch, André, Baumier, Vincent, Bayer, Guillaume, Bayle, Sophie, Beauvais, Catherine, Beinat, Rudie, Belin, Véronique, Belkhir, Rakiba, Benainous, Ruben, Belot, Alexandre, Benammar, Mohammed, Benhamou, Mathilde, Benhamou, Ygal, Benmansour, Ahmed, Bennet, Pascal, Bernoux-Manat, Brigitte, Berthet, Elise, Berthoux, Emilie, Bertolini, Ewa, Bigot, Adrien, Bisson-Vaivre, Aurélia, Blaison, Gilles, Bolla, Gilles, Bonidan, Olivier, Bonnet, Christine, Borie, Raphaël, Bossert, Marie, Boudou, Laurence, Bouhour, Françoise, Bouiller, Kévin, Bouldoires, Bastien, Boussoualim, Karima, Bouvard, Eric, Brondino, Regine, Buchlin, Pierre, Cabantous, Laurence, Cacoub, Patrice, Cadiou, Simon, Carteni, Maurizio, Carbasse, Aurélia, Castel, Brice, Cathebras, Pascal, Caumont, Hervé, Celant, Annalisa, Chaigne, Benjamin, Chaillous, Benoît, Champy, Romuald, Charcot, Agnès, Charles, Pierre, Charlot-Lambrecht, Isabelle, Charpin, Caroline, Chatelus, Emmanuel, Chaudier, Bernard, Chertok, Pascale, Chevalier, Xavier, Chevreau, Maxime, Chotard, Emilie, Chu Miow Lin, Delphine, Clavel, Gaëlle, Clavel-Osorio, Cyril, Cohen, Fleur, Cohen, Gregory, Colette-Cedoz, Marie-Eve, Collercandy, Nived, Colombey, Antoine, Comarmond, Chloé, Combe, Bernard, Comparon, Céline, Constant, Elodie, Coquerelle, Pascal, Corli, Justine, Corre, Clémence, Costedoat-Chalumeau, Nathalie, Couret, Marie, Courvoisier, Natacha, Coury-Lucas, Fabienne, Coutarel, Cécile, Coutier, Fabrice, Damade, Richard, Daver-Malaterre, Laurence, Dehimat, Sarahe, Delahousse, Michel, Barrois-Delattre, Emilie, Denarie, Delphine, Deprouw, Camille, Dernis, Emanuelle, Deroux, Alban, Desbarbieux, Renaud, Descamps, Elise, Deslandre, Chantal, Desmurs, Marie, Despaux, Jacques, Desplats, Marie, Detree, Frédérick, Devauchelle-Pensec, Valérie, Dhote, Robin, Dieude, Philippe, Dieudonne, Yannick, Diot, Elisabeth, Direz, Guillaume, Djeddi, Djamal-Dine, Douvier, Sarah, Drouet, Béatrice, Drumez, Elodie, Duc, Catherine, Ducornet, Angélique, Dufauret-Lombard, Carine, Duhamel, Alain, Dumaine, Cécile, Dumel, Anne-Elisabeth, Dumoulin-Richez, Chantal, Duquesne, Agnès, Durand, Géraldine, Durandin-Truffinet, Mariane, Duret, Pierre-Marie, Duval, Maïka, Ebbo, Mikaël, Ebstein, Esther, Economu-Dubosc, Andra, Emilie, Stéphanie, Euvrard, Romain, Evon, Philippe, Fabre, Sylvie, Fagedet, Dorothée, Farhat, Meryem, Fauconier, Marion, Fechtenbaum, Jacques, Felten, Renaud, Fernandes, Fanny, Ferreira-Maldent, Nicole, Feurer, Elodie, Fichet, Amandine, Flaisler, Françoise, Florens, Nans, Foltz, Violaine, Fontanges, Elisabeth, Foret, Jennifer, Fougerousse, Anne-Claire, Fouque-Aubert, Anne, Foutrier-Morello, Catherine, Francois-Pradier, Hélène, Frantzen, Léa, Fritz, Pierre, Froissart, Antoine, Fulpin, Jean, Fuzibet, Piera, Gaches, Francis, Gagneux-Lemoussu, Laurence, Penhoat-Gahier, Mélanie, Galland, Joris, Gandjbakhch, Frédérique, Garnier, Nicole, Garraud, Thomas, Garrot, Jean-François, Gastaldi, Romain, Gaud-Listrat, Véronique, Gauthier-Prieur, Maud, Georgescu, Dana, Gerard, Nathalie, Gervais, Elisabeth, Gibert, Christelle, Gibert, Eric, Gill, Ghislaine, Gillard, Jérôme, Gilson, Mélanie, Gimonnet, Pauline, Giraudet-Le Quintrec, Jeanine-Sophie, Giraud-Morelet, Aude, Glace, Baptiste, Glanowski, Camille, Godeau, Bertrand, Gombert, Bruno, Gonnet-Gracia, Camille, Goulenok, Tiphaine, Goupille, Philippe, Gourmelen, Olivier, Govindaraju-Audouard, Sophie, Grados, Franck, Grall-Lerosey, Martine, Grardel, Bruno, Grasland, Anne, Grateau, Gilles, Groza, Monica, Guillaud, Constance, Guillaume, Séverine, Guillibert, Caroline, Guillot, Xavier, Guilpain, Philippe, Gury, Aline, Guyot, Marie-Hélène, Hacquard-Bouder, Cécile, Havard, Marie-Noelle, Hellier, Jean-Pierre, Hennequin, Pascal, Henriot, Basile, Henry, Julien, Hentgen, Véronique, Hermet, Marion, Herasse, Muriel, Hernandez, Julie, Hie, Miguel, Hilliquin, Pascal, Hinschberger, Olivier, Hittinger-Roux, Ambre, Holubar, Jan, Hudry, Christophe, Huguenel, Serge, Jaccard, Clara, Jacquemier, Jean-Michel, Jamard, Bénédicte, Jan, Catherine, Jean, Sylvie, Jouvray, Mathieu, Juge, Pierre-Antoine, Juillard, Laurent, Jullien, Denis, Kabala, Anna, Kabchou, Abdelkrim, Karkowski, Ludovic, Karman, Françoise, Kemiche, Farid, Keraen, Jérémy, Kieffer, Pierre, Kone-Paut, Isabelle, Koreichi, Abdeldajallil, Kostine, Marie, La Batide Alanore, Sylvain, Lafforgue, Pierre, Lahalle, Sophie, Lambert, Marc, Lambrecht, Isabelle, Lanot, Sylvain, Lanteri, Aurélia, Larbre, Jean-Paul, Latourte, Augustin, Lavigne, Christian, Le Guen Guegan, Sophie, Le Guenno, Guillaume, Leguy, Diane, Lebrun, Agnès, Ledoult, Emmanuel, Legoupil, Nathalie, Legrand, Erick, Lejeune, Charlotte, Leloire, Olivier, Leroux, Christophe, Leroy, Rémi, Leroy-Gouix, Marie, Leturcq, Tifenn, Leurs, Amélie, Leveque-Michaud, Céline, Limbach, François-Xavier, Liote, Frédéric, Lohse, Anne, Lozac'h, Pierre, Lucas, Virginie, Madelon, Aurélie, Magy-Bertrand, Nadine, Mahevas, Matthieu, Maillard, Hélène, Maillet, Thibault, Malochet-Guinamand, Sandrine, Mangon, Quentin, Marchou-Lopez, Sylvie, Margarit, Nathalie, Marhadour, Thierry, Mariette, Xavier, Martin, Claire, Mathian, Alexis, Maurier, François, Maury, Frédéric, Mazet-Guillaume, Betty, Mazouyez, Arnaud, Mazyad, Hassan, Mehsen-Cetre, Nadia, Meinzer, Ulrich, Melki, Isabelle, Messer, Laurent, Miceli, Corinne, Michaud, Martin, Michel, Catherine, Michel, Matthias, Michon, Mathilde, Milesi-Lecat, Anne-Marie, Molto, Anna, Moly, Marie, Moranne, Olivier, Morel, Gautier, Morel, Hugo, Morel, Jacques, Morin, Franck, Moulinier, Laurence, Moulis, Guillaume, Moura, Bertrand, Nguyen, Minh, Nicolas-Vullierme, Sabine, Nielly, Hubert, Nocturne, Gaétane, Nottez, Aurore, Ollagnon, Henri-Olivier, Pacaud-Vitoux, Isabelle, Pagnier, Anne, Paris, Caroline, Parrot, Antoine, Pascart, Tristan, Pascaud-Mansour, Yasmina, Paulin, Lætitia, Pavy, Stephan, Perard, Laurent, Pers, Yves-Marie, Pha, Micheline, Pichon, Maud, Pierreisnard, Audrey, Pizana, Gabrielle, Poignant, Sylvaine, Poix, Elsa, Portier, Agnès, Poulet, Antoine, Plassard, Samira, Pugnet, Grégory, Puyraimond-Zemmour, Déborah, Quartier-Dit-Maire, Pierre, Quenet, Marion, Queyrel, Viviane, Raffray, Loïc, Remy, Philippe, Renard, Myriam, Rene, Jessica, Revuz, Sabine, Rey, Bénédicte, Richard-Colmant, Gaëlle, Riviere, Etienne, Riviere, Sébastien, Robin, Sophie, Rohmer, Julien, Roitg, Isabelle, Romier, Mélanie, Rolland, Michel, Roriz, Mélanie, Rosenberg, Carole, Rossi, Linda, Roth, Olivier, Rouidi, Sid-Ahmed, Roumier, Mathilde, Rousiere, Mickaël, Rousselin, Clémentine, Rouviere, Bénédicte, Roux, Christian, Roux, Fabienne, Roux, Marielle, Roux, Nicolas, Rouzaud, Diane, Rozenberg, Sylvie, Sacco, Isabelle, Sadji, Fatiha, Sailler, Laurent, Salliot, Carine, Salmon, Jean-Hugues, Saraux, Alain, Schmidt, Jean, Seguier, Julie, Sellam, Jérémie, Senbel, Eric, Sene, Thomas, Senet, Patricia, Seve, Pascal, Sicaud, Aurélie, Smets, Perrine, Sobanski, Vincent, Sordet, Christelle, Sornay-Rendu, Elisabeth, Souchaud-Debouverie, Odile, Sparsa, Lætitia, Spielmann, Lionel, Steib, Sarah, Stavris, Chloé, Straus, Catherine, Strotz, Victor, Szafors, Paulina, Taffignon-Clave, Séverine, Simoens, Justine, Theillac, Claire, Tenenbaum, Nora, Thomachot, Benoît, Tieulie, Nathalie, Tiriau, Soizic, Tison, Alice, Toussirot, Eric, Trefond, Ludovic, Trijau, Sophie, Trouillier, Sébastien, Trouvin, Anne-Priscille, Truchetet, Marie-Elise, Ulrich, Marc, Vaquier, Jacques, Veillard, Eric, Veillon, Laurent, Vial, Guillaume, Viallard, Jean-François, Victor, Judith, Vidon, Claire, Vidon, Mathias, Vigne, Camille, Virone, Alexandre, Warzocha, Ursula, Wendling, Daniel, Werle, Claude, Wibaux, Cécile, Willems, Alexandra, Wisniewski, Michel, Woessner, Juliette, Xerri-Campano, Bernadette, Avouac, Jérôme, Hachulla, Eric, Seror, Raphaèle, Georgin-Lavialle, Sophie, El Mahou, Soumaya, Pertuiset, Edouard, Pham, Thao, Marotte, Hubert, Servettaz, Amélie, Domont, Fanny, Chazerain, Pascal, Devaux, Mathilde, Claudepierre, Pascal, Langlois, Vincent, Mekinian, Arsène, Maria, Alexandre Thibault Jacques, Banneville, Béatrice, Fautrel, Bruno, Pouchot, Jacques, Thomas, Thierry, Flipo, René-Marc, and Richez, Christophe

Published
2021
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5. Vasculitis associated with myelodysplastic syndrome and chronic myelomonocytic leukemia: French multicenter case-control study

Author

Roupie, Anne Laure, Guedon, Alexis, Terrier, Benjamin, Lahuna, Constance, Jachiet, Vincent, Regent, Alexis, de Boysson, Hubert, Carrat, Fabrice, Seguier, Julie, Terriou, Louis, Versini, Mathilde, Queyrel, Viviane, Groh, Matthieu, Benhamou, Ygal, Maurier, Francois, Ledoult, Emmanuel, Clech, Lenaig Le, D'Aveni, Maud, Rossignol, Julien, Galland, Joris, Willems, Lise, Chiche, Noemie Jourde, Peterlin, Pierre, Roux-Sauvat, Marielle, Parcelier, Anne, Wemeau, Matthieu, Lambert, Marc, Belizna, Cristina, Puechal, Xavier, Swiader, Laure, Cohen-Valensi, Rolande, Noc, Valérie, Dao, Emmanuel, Thepot, Sylvain, de Frémont, Grégoire Martin, Tanguy-Schmidt, Aline, Koka, Anne Marfaing, Bussone, Guillaume, Philipponnet, Carole, Konate, Amadou, Cavaille, Guilhem, Guilpain, Philippe, Allain, Jean-Sébastien, Broner, Jonathan, Solary, Eric, Ruivard, Marc, de Renzis, Benoit, Corm, Sélim, Baati, Nadia, Schleinitz, Nicolas, Ponsoye, Matthieu, Stamatoullas-Bastard, Aspasia, Ades, Lionel, Dellal, Azeddine, Tchirkov, Andrei, Aouba, Achille, Fenaux, Pierre, Fain, Olivier, and Mekinian, Arsène

Published
2020
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7. Clinical presentation, treatment and outcome of IgG4-related pachymeningitis: From a national case registry and literature review

Author

Melenotte, Cléa, Seguier, Julie, Ebbo, Mikael, Kaphan, Elsa, Bernit, Emmanuelle, Saillier, Laurent, Audoin, Bertrand, Feyeux, Delphine, Daniel, Laurent, Roche, Pierre-Hugues, Graillon, Thomas, Dufour, Henry, Boutière, Clémence, Girard, Nadine, Closs-Prophette, Fabienne, Guillaud, Constance, Tieulié, Nathalie, Regent, Alexis, Harlé, Jean Robert, Hamidou, Mohamed, Mekinian, Arsène, Grados, Aurélie, and Schleinitz, Nicolas

Published
2019
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11. Seven cases of hereditary haemorrhagic telangiectasia-like hepatic vascular abnormalities associated with EPHB4 pathogenic variants.

Author

Guilhem, Alexandre, Dupuis-Girod, Sophie, Espitia, Olivier, Rivière, Sophie, Seguier, Julie, Kerjouan, Mallorie, Lavigne, Christian, Maillard, Hélène, Magro, Pascal, Alric, Laurent, Lipsker, Dan, Parrot, Antoine, Leguy, Vanessa, Vanlemmens, Claire, Guibaud, Laurent, Vikkula, Miikka, Eyries, Melanie, Valette, Pierre-Jean, and Giraud, Sophie

Abstract

Background EPHB4 loss of function is associated with type 2 capillary malformation–arteriovenous malformation syndrome, an autosomal dominant vascular disorder. The phenotype partially overlaps with hereditary haemorrhagic telangiectasia (HHT) due to epistaxis, telangiectases and cerebral arteriovenous malformations, but a similar liver involvement has never been described. Methods Members of the French HHT network reported their cases of EPHB4 mutation identified after an initial suspicion of HHT. Clinical, radiological and genetic characteristics were analysed. Results Among 21 patients with EPHB4, 15 had a liver imaging, including 7 with HHT-like abnormalities (2 female patients and 5 male patients, ages 43–69 years). Atypical epistaxis and telangiectases were noted in two cases each. They were significantly older than the eight patients with normal imaging (median: 51 vs 20 years, p<0.0006). The main hepatic artery was dilated in all the cases (diameter: 8–11mm). Six patients had hepatic telangiectases. All kind of shunts were described (arteriosystemic: five patients, arterioportal: two patients, portosystemic: three patients). The overall liver appearance was considered as typical of HHT in six cases. Six EPHB4 variants were classified as pathogenic and one as likely pathogenic, with no specific hot spot. Conclusion EPHB4 loss-of-function variants can be associated with HHT-like hepatic abnormalities and should be tested for atypical HHT presentations. [ABSTRACT FROM AUTHOR]

Published
2023
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12. Tocilizumab versus anakinra in COVID-19: results from propensity score matching.

Author

Arcani, Robin, Correard, Florian, Suchon, Pierre, Kaplanski, Gilles, Jean, Rodolphe, Cauchois, Raphael, Leprince, Marine, Arcani, Vincent, Seguier, Julie, De Sainte Marie, Benjamin, Andre, Baptiste, Koubi, Marie, Rossi, Pascal, Gayet, Stéphane, Gobin, Nirvina, Garrido, Victoria, Weiland, Joris, Jouve, Elisabeth, Couderc, Anne-Laure, and Villani, Patrick

Subjects
SARS-CoV-2, PROPENSITY score matching, CORONAVIRUS diseases, ANAKINRA, COVID-19
Abstract

Background: Tocilizumab and anakinra are anti-interleukin drugs to treat severe coronavirus disease 2019 (COVID-19) refractory to corticosteroids. However, no studies compared the efficacy of tocilizumab versus anakinra to guide the choice of the therapy in clinical practice. We aimed to compare the outcomes of COVID-19 patients treated with tocilizumab or anakinra. Methods: Our retrospective study was conducted in three French university hospitals between February 2021 and February 2022 and included all the consecutive hospitalized patients with a laboratory-confirmed severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection assessed by RTPCR who were treated with tocilizumab or anakinra. A propensity score matching was performed to minimize confounding effects due to the non-random allocation. Results: Among 235 patients (mean age, 72 years; 60.9% of male patients), the 28-day mortality (29.4% vs. 31.2%, p = 0.76), the in-hospital mortality (31.7% vs. 33.0%, p = 0.83), the high-flow oxygen requirement (17.5% vs. 18.3%, p = 0.86), the intensive care unit admission rate (30.8% vs. 22.2%, p = 0.30), and the mechanical ventilation rate (15.4% vs. 11.1%, p = 0.50) were similar in patients receiving tocilizumab and those receiving anakinra. After propensity score matching, the 28-day mortality (29.1% vs. 30.4%, p = 1) and the rate of high-flow oxygen requirement (10.1% vs. 21.5%, p = 0.081) did not differ between patients receiving tocilizumab or anakinra. Secondary infection rates were similar between the tocilizumab and anakinra groups (6.3% vs. 9.2%, p = 0.44). Conclusion: Our study showed comparable efficacy and safety profiles of tocilizumab and anakinra to treat severe COVID-19. [ABSTRACT FROM AUTHOR]

Published
2023
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13. Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients.

Author

Khitri, Mohamed-Yacine, Guedon, Alexis F., Georgin-Lavialle, Sophie, Terrier, Benjamin, Saadoun, David, Seguier, Julie, le Besnerais, Maelle, De Moreuil, Claire, Denis, Guillaume, Gerfaud-Valentin, Mathieu, Allain, Jean Sebastien, Maria, Alexandre, Bouillet, Laurence, Grobost, Vincent, Galland, Joris, Kosmider, Olivier, Dumont, Anael, Devaux, Mathilde, Subran, Benjamin, and Schmidt, Jean

Published
2022
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14. Acute severe hepatitis in adult-onset Still's disease: case report and comprehensive review of a life-threatening manifestation.

Author

Muller, Romain, Briantais, Antoine, Faucher, Benoit, Borentain, Patrick, Nafati, Cyril, Blasco, Valery, Gregoire, Emilie, Bernit, Emmanuelle, Seguier, Julie, Meunier, Benoit, Harlé, Jean-Robert, Ebbo, Mikael, and Schleinitz, Nicolas

Subjects
HEPATITIS, REPORTING of diseases, MEDICAL literature, LIVER biopsy, LIVER transplantation
Abstract

Acute severe hepatitis is a rare complication of adult-onset Still's disease (AOSD). This condition is poorly characterized. We performed a review of the medical literature to describe clinical, biological, pathological, and treatment characteristics from AOSD patients with acute severe hepatitis. Their characteristics were compared with AOSD patients without severe hepatitis. Twenty-one cases were collected including a new case reported here. Patients with severe hepatitis were mostly young adults with a median age of 28 years (range: 20 to 55 years). Overall, patients with severe hepatitis had less arthritis, macular rash, sore throat, lymphadenopathy, or splenomegaly than patients without severe hepatitis. Cytopenia was more frequent in case of severe hepatitis. Most patients were treated with steroids, and the use of biotherapies has increased over the last decade. Despite treatment, 49% of patients required liver transplantation and 24% died. Key Points • Acute severe hepatitis in adult-onset Still's disease (AOSD) is associated with liver transplantation and/or death in, respectively, 43% and 24% of cases. • Severe hepatitis is the inaugural manifestation of AOSD in half of cases. Diagnosis is difficult when extra-hepatic clinical manifestations are lacking. • The mechanism of hepatic necrosis in AOSD with severe hepatitis is unknown. Liver biopsy is not specific and should not delay treatment initiation. [ABSTRACT FROM AUTHOR]

Published
2021
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15. Paradoxical association between blood modular interferon signatures and quality of life in patients with systemic lupus erythematosus.

Author

Seguier, Julie, Jouve, Elisabeth, Bobot, Mickaël, Whalen, Elisabeth, Dussol, Bertrand, Gentile, Stéphanie, Burtey, Stéphane, Halfon, Philippe, Retornaz, Frédérique, Chaussabel, Damien, Chiche, Laurent, and Jourde-Chiche, Noémie

Subjects
*HEALTH status indicators, *HEALTH surveys, *INTERFERONS, *MENTAL health, *MULTIVARIATE analysis, *QUALITY of life, *QUESTIONNAIRES, *SYSTEMIC lupus erythematosus, *LUPUS nephritis, *GENE expression profiling, *DESCRIPTIVE statistics
Abstract

Objectives Blood transcriptomic IFN signature is a hallmark of SLE. The impaired health-related quality of life (HRQOL) observed in SLE is poorly related to disease activity. The aim of this study was to test how IFN signatures were associated with HRQOL in SLE patients. Methods Among consecutive patients, blood transcriptomic profiles were analysed with a modular framework comprising 3 IFN modules: M1.2, M3.4 and M5.12. Disease activity was evaluated by the SLEDAI score, and HRQOL was assessed with the SF-36 questionnaire, which includes eight domains: physical function, role physical, bodily pain, general health, vitality, social functioning, role emotional, and mental health (MH) and physical component summary and mental component summary scores. Results A total of 57 SLE patients were evaluated, among whom 27 (47%) were clinically quiescent, 30 (53%) were flaring, and 19 (33%) had active lupus nephritis. All SF-36 domains were altered in SLE patients compared with the general French population (P < 0.0001). In multivariate analysis, taking into account flares, age, ethnicity, smoking and renal severity, social functioning was independently associated with the IFN score (P = 0.027). Analyses restrained to quiescent patients (n = 27) yielded greater associations between social functioning and the three IFN modules, and between MH and M3.4. Considering all quiescent visits (n = 51), the IFN score was independently correlated with social functioning (P = 0.022) and MH (P = 0.038). Conclusion This unexpected paradoxical association between IFN signature and some specific HRQOL domains argues against a pivotal role of IFNs in the persistently altered HRQOL of SLE patients. [ABSTRACT FROM AUTHOR]

Published
2020
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16. Life-threatening autoimmune warm hemolytic anemia following treatment for multiple sclerosis with alemtuzumab.

Author

Meunier, Benoit, Rico, Audrey, Seguier, Julie, Boutiere, Clemence, Ebbo, Mikael, Harle, Jean Robert, Schleinitz, Nicolas, and Pelletier, Jean

Subjects
AUTOIMMUNE diseases, HEMOLYTIC anemia, MULTIPLE sclerosis, ALEMTUZUMAB, MONOCLONAL antibodies
Abstract

Background: Alemtuzumab is a humanized monoclonal antibody directed at CD52 approved as a disease-modifying therapy for relapsing forms of multiple sclerosis (MS). Objective: To describe a case of a life-threatening autoimmune anemia occurring after a first course of alemtuzumab for relapsing-remitting MS in a 28-year-old male. Methods: Case report. Results: A 28-year-old male developed a life-threatening autoimmune anemia occurring 11 months after first alemtuzumab course. Conclusion: We report the third case of autoimmune hemolytic anemia following treatment with alemtuzumab in a young MS patient. Due to the severity of this adverse event, neurologists using this treatment should be alert. [ABSTRACT FROM AUTHOR]

Published
2018
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17. 0082: Multimodality imaging in cardiac amyloidosis: respective contributions of echocardiography, MRI and scintigraphy.

Author

Maysou, Laurie-Anne, Seguier, Julie, Fernandez, Rémi, Flavian, Antonin, Tessonnier, Laetitia, Saby, Ludivine, Michel, Nicolas, Hubert, Sandrine, Sumian, Marion, Salaun, Erwann, Renard, Sebastien, Avierinos, Jean-François, Verschueren, Annie, Franques, Jérôme, Mancini, Julien, Mundler, Olivier, Pouget, Jean, Jacquier, Alexis, Serratrice, Jacques, and Habib, Gilbert

Abstract

Context Amyloidosis(A) prognosis is determined by cardiac involvement.The main types of A are immunoglobulin light chain(AL) and transthyretin-related(TTR), which can be mutated(TTRm) or senile(TTRwt). Specific treatments can’t be administrated unless A has been typed histologically.Literature suggests echocardiography, 99m Tc DPD scintigraphy and cardiac MRI could help typing A. We described these imaging modalities to assess these potential tools for an uninvasive typing. Material and methods We analysed these imaging modalities in patients examined at Cardiomyopathies Competence Center(CCC) of La Timone Hospital in Marseille, with an histologically proven diagnosis of cardiac A(CA). Results We included 75patients examined between September 2006 and March 2014 at CCC, with a strongly suspected diagnosis of CA.CA could be histologically confirmed and typed in 45 patients(10 TTRm, 4 TTRwt, 6 TTR undeterminated; 19 AL;6 of other type).In 11 patients, CA was confirmed but untyped.No statistically significant difference was found between TTR and AL patients for the various imaging modalities.We observed 71% of men, aged 66, NYHA stage 2,4 on average.In all patients, cardiac biomarkers rates were increased. Myocardic mass and interventricular septal thickness were increased (199g/m² and 19mm), restrictive filling pattern was observed in 83% of patients.Despite a relatively preserved left ventricular ejection fraction, Global Longitudinal Strain was impaired at – 11%, with an apical sparing. Scintigraphy showed a frequent myocardic fixation (69%), slightly more intense in TTR patients.Cardiac MRI showed a constant late gadolinium enhancement, more extended in AL patients. Conclusion We didn’t observe the differences described between CA types, probably because of a lack of statistical power.This encouraged us to develop a protocol for multidisciplinary evaluation of CA, to improve the management of this disease, and to keep on evaluating the diagnostic accuracy of these imaging modalities. [ABSTRACT FROM AUTHOR]

Published
2015
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18. Giant-cell arteritis associated with myelodysplastic syndrome: French multicenter case control study and literature review.

Author

Roupie, Anne Laure, de Boysson, Hubert, Thietart, Sara, Carrat, Fabrice, Seguier, Julie, Terriou, Louis, Versini, Mathilde, Queyrel, Viviane, Groh, Matthieu, Benhamou, Ygal, Maurier, Francois, Decaux, Olivier, d'Aveni, Maud, Rossignol, Julien, Galland, Joris, Solary, Eric, Willems, Lise, Schleinitz, Nicolas, Ades, Lionel, and Dellal, Azeddine

Subjects
*GIANT cell arteritis, *MYELODYSPLASTIC syndromes, *LITERATURE reviews, *ARTERITIS, *TREATMENT effectiveness, *CHRONIC leukemia
Abstract

Myelodysplastic syndromes (MDS) and MDS/myeloproliferative neoplasms (MDS/MPN) can be associated with giant cell arteritis (GCA). In this nationwide study by the "French Network of dysimmune disorders associated with hemopathies" (MINHEMON) the objective was to evaluate characteristics, treatment and outcome of GCA MDS-MDS/MPN. Retrospective analysis of patients that presented a MDS or MDS/MPN associated with GCA. Treatment efficiency, relapse-free and overall survival of GCA MDS-MDS/MPN were compared to GCA alone. Twenty-one patients with GCA MDS-MDS/MPN were included with median age 76 [42–92], M/F ratio 2.5, 8 MDS with multilineage dysplasia (38%), 4 chronic myelomonocytic leukemia (19%), at low or intermediate risk according to IPPS and IPSS-R. The prevalence of headaches, jaw claudication and anterior ischemic optic neuropathy was significantly lower in patients with GCA MDS-MDS/MPN compared to idiopathic GCA (14.3%, 0% and 0% versus 30%, 25%, and 25%, respectively; p <.05). Other clinical and histology findings were similar. All GCA patients received steroid therapy as first-line treatment. Complete or partial response was observed in 14 GCA MDS-MDS/MPN patients (66.7%), of whom 6 (28.6%) received combined immunosuppressive therapies (versus 10% of idiopathic GCA; p =.07). Relapse incidence was similar in the two groups. Steroid dependence was more frequent among GCA MDS-MDS/MPN patients (12 (57%) versus 18 (22.5%); p <.05). Relapse-free and steroid-free survivals were significantly decreased in GCA MDS-MDS/MPN patients (log rank 0.002 and 0.049 respectively), but not overall survival. Characteristics of GCA MDS-MDS/MPN seem different than idiopathic GCA, with a distinct clinical phenotype and poorer outcome with a higher risk of steroid dependence and relapse. [ABSTRACT FROM AUTHOR]

Published
2020
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