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1. Data-driven subclassification of ANCA-associated vasculitis: model-based clustering of a federated international cohort

Author

Tassoni, Adrian, Mohammad, Aladdin J, Bettiol, Alessandra, Tais, Arlette, White, Arthur, Vaglio, Augusto, Terrier, Benjamin, Yaman, Beyza, Armstrong, Cecil, O'Sullivan, Declan, Wandrei, Dagmar, Kalra, Dipak, Schubach, Fabian, Dradin, François, Emmi, Giacomo, Bagni, Giacomo, Ihorst, Gabriele, Aerts, Hannelore, Kardaoui, Hicham, Mattioli, Irene, Sengers, Iris, Musial, Jacek, Scott, Jennifer, Mills, John, Julie Power, Julie, Gisslander, Karl, Wawrzycka-Adamczyk, Katarzyna, McGlinn, Kris, Wójcik, Krzysztof, Aslett, Louis, Hederman, Lucy, Dunne, Margaret, Alba, Marco A, Christofidou, Maria, Little, Mark A, Segelmark, Mårten, Crnogorac, Matija, Rutherford, Matthew, Tesi, Michelangelo, Lea, Nathan, Basu, Neil, Lamprecht, Peter, Verhoeven, Peter, de Boer, Raïssa, Straka, Richard, Lichołai, Sabina, Arnold, Sabrina, Tesar, Vladimir, Puéchal, Xavier, Hruskova, Zdenka, Hrušková, Zdenka, Musiał, Jacek, Nazeer, Jamsheela, Ng, James, and Tesař, Vladimir

Published
2024
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2. French protocol for the diagnosis and management of systemic lupus erythematosus

Author

Andre, Marc, Bienvenu, Boris, Blaison, Gilles, Blanco, Patrick, Cathebras, Pascal, Chauveau, Dominique, Chosidow, Olivier, Clouscard, Johanna, Deligny, Christophe, Duhaut, Pierre, Gobert, Pierre, Levesque, Hervé, Magy-Bertrand, Nadine, Melki, Isabelle, Pha, Micheline, Puechal, Xavier, Queyrel, Viviane, Raffray, Loïc, Ranchin, Bruno, Riviere, Marianne, Roblot, Pascal, Servettaz, Amelie, Amoura, Zahir, Bader-Meunier, Brigitte, Antignac, Marie, Bardin, Nathalie, Belizna, Cristina, Belot, Alexandre, Bonnotte, Bernard, Bouaziz, Jean-David, Chasset, François, Chiche, Laurent, Cohen, Fleur, Costedoat-Chalumeau, Nathalie, Daugas, Eric, Devilliers, Hervé, Diot, Elisabeth, Elefant, Elisabeth, Faguer, Stanislas, Ferreira, Nicole, Hachulla, Eric, Hanslik, Thomas, Hie, Miguel, Jourde-Chiche, Noémie, Le Guern, Véronique, Martin, Thierry, Mathian, Alexis, Michel, Marc, Miyara, Makoto, Papo, Thomas, Richez, Christophe, Scherlinger, Marc, Sibilia, Jean, Uzunhan, Yurdagul, Wahl, Denis, Wojtasik, Géraldine, and Yelnik, Cécile

Published
2024
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4. Glucocorticoids versus glucocorticoids plus cyclophosphamide in eosinophilic granulomatosis with polyangiitis with poor-prognosis factors

Author

Sorin, Boris, Papo, Matthias, Sinico, Renato A., Teixeira, Vítor Silvestre, Venhoff, Nils, Urban, Maria-Letizia, Iudici, Michele, Mahrhold, Juliane, Locatelli, Francesco, Cassone, Giulia, Schiavon, Franco, Seeliger, Benjamin, Neumann, Thomas, Feder, Claudia, Kroegel, Claus, Groh, Matthieu, Marvisi, Chiara, Samson, Maxime, Barba, Thomas, Jayne, David, Troilo, Arianna, Thiel, Jens, Hellmich, Bernhard, Monti, Sara, Montecucco, Carlomaurizio, Salvarani, Carlo, Kahn, Jean-Emmanuel, Bonnotte, Bernard, Durel, Cécile-Audrey, Puéchal, Xavier, Mouthon, Luc, Guillevin, Loïc, Emmi, Giacomo, Vaglio, Augusto, Porcher, Raphael, and Terrier, Benjamin

Published
2024
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6. Diagnosis and treatment of Tropheryma whipplei infection in patients with inflammatory rheumatic disease: Data from the French Tw-IRD registry

Author

Caillet Portillo, Damien, Puéchal, Xavier, Masson, Maëva, Kostine, Marie, Michaut, Alexia, Ramon, André, Wendling, Daniel, Costedoat-Chalumeau, Nathalie, Richette, Pascal, Marotte, Hubert, Vix-Portet, Justine, Dubost, Jean-Jacques, Ottaviani, Sébastien, Mouterde, Gaël, Grasland, Anne, Frazier, Aline, Germain, Vincent, Coury, Fabienne, Tournadre, Anne, Soubrier, Martin, Cavalie, Laurent, Brevet, Pauline, Zabraniecki, Laurent, Jamard, Bénédicte, Couture, Guillaume, Arnaud, Laurent, Richez, Christophe, Degboé, Yannick, Ruyssen-witrand, Adeline, and Constantin, Arnaud

Published
2024
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9. Clinical features and long-term outcomes of patients with systemic polyarteritis nodosa diagnosed since 2005: Data from 196 patients

Author

Ackermann, Felix, Aumaitre, Olivier, Bussone, Guillaume, Catalan, Pilartxo, Chasset, François, Crabol, Yoann, de Moreuil, Claire, Hot, Arnaud, Humbert, Marc, Chiche, Noémie Jourde, Le Gallou, Thomas, Leroux, Dominique, Mesbah, Rafik, Mouthon, Luc, Pagnoux, Christian, Revuz, Sabine, Rieu, Laurent, Schmidt, Jean, Vandergheynst, Frederic, Rohmer, Julien, Nguyen, Yann, Trefond, Ludovic, Agard, Christian, Allain, Jean Sebastien, Berezne, Alice, Charles, Pierre, Cohen, Pascal, Gondran, Guillaume, Groh, Matthieu, Huscenot, Tessa, Lacout, Carole, Lazaro, Estibaliz, London, Jonathan, Maurier, François, Mekinian, Arsène, Nubourgh, Isabelle, Perard, Laurent, Puéchal, Xavier, Pugnet, Gregory, Puyade, Mathieu, Queyrel, Viviane, Roux, Arthur, Rouzaud, Diane, Durel, Cecile-Audrey, Guillevin, Loïc, and Terrier, Benjamin

Published
2023
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10. Pregnancy outcomes in women with primary Sjögren's syndrome: an analysis of data from the multicentre, prospective, GR2 study

Author

AUDEMARD-VERGER, Alexandra, AZZI, Emmanuel, BANNEVILLE, Béatrice, BAUDET, Antoine, BEAUDOUIN BAZIRE, Constance, BELIZNA, Cristina, Belot, Alexandre, BENHAMOU, Ygal, Berezné, Alice, BERNARD-GUERVILLY, Fanny, BERTHIER, Sabine, BEZANAHARY, Holy, BIALE, Lisa, BIGOT, Adrien, BLANCHARD-DELAUNAY, Claire, CALAS, Anne, CAMPAGNE, Julien, CATHEBRAS, Pascal, CAZALETS, Claire, CHAIGNE, Benjamin, CHANDESRIS, Olivia, CHATELAIS, Jérémy, CHATELUS, Emmanuel, COHEN, Fleur, Combe, Bernard, COMPARON, Céline, COQUERELLE, Pascal, DAMIAN, Louise, DAUGAS, Eric, DE MENTHON, Mathilde, DE MOREUIL, Claire, DELATTRE, Estelle, DELLAL, Azeddine, Deneux-Tharaux, Catherine, DENIS, Amélie, DEPROUW, Camille, DERNIS, Emmanuelle, DEROUX, Alban, DESOUCHES, Sandra, Dieudé, Philippe, DIREZ, Guillaume, Dougados, Maxime, DRIESSEN, Marine, DU THANH, Aurélie, DUNOGEANT, Laetitia, DURANT, Cécile, DUREL, Cécile-Audrey, DURIEU, Isabelle, EBOUE, Florence, Elefant, Elisabeth, FAIN, Olivier, FAUTREL, Bruno, FLIPO, René-Marc, FRAZIER, Aline, FROISSART, Antoine, GEORGIN-LAVIALLE, Sophie, GERVAIS, Elisabeth, GODEAU, Bertrand, Goffinet, François, GOMPEL, Anne, GOSSEC, Laure, GOUPILLE, Philippe, GRANGE, Claire, GUILLAUD-DANIS, Constance, HACHULLA, Eric, HOEFSLOOT, Sabine, HUMMEL, Aurélie, JEGO, Patrick, JOBARD, Stéphanie, JOSSELIN-MAHR, Laurence, LAMBERT, Marc, LANGLOIS, Vincent, LARIVIERE, Delphine, LARROCHE, Claire, LATOURTE, Augustin, LAVIGNE, Christian, LE GALLOU, Thomas, LEROUX, Gaëlle, LETAROUILLY, Jean Guillaume, LIOTÉ, Frédéric, Loeuillet, Laurence, London, Jonathan, Loustau, Valentine, LOZAC'H, Pierre, MAHEU, Emmanuel, MAILLARD, Hélène, MAROTTE, Hubert, MASSEAU, Agathe, MEKINIAN, Arsène, Melboucy Belkhir, Sara, Miceli-Richard, Corinne, MICHAUD, Martin, MICHEL, Marc, MORANNE, Olivier, MORATI-HAFSAOUI, Chafika, MOULIS, Guillaume, MOUTHON, Luc, NICOLAS, Barbara, Nizard, Jacky, ORA, Jérémy, OUTH, Rodérau, PASQUIER, Elisabeth, PENNAFORTE, Jean-Loup, PERLAT, Antoinette, PETIT-BAUER, Hélène, PILLEBOUT, Evangeline, PIOT, Jean-Maxime, PORTIER, Agnès, Pourrat, Olivier, PUECHAL, Xavier, PUGNET, Gregory, REDONDIN, Manon, REGENT, Alexis, RORIZ, Mélanie, SAILLER, Laurent, SAVEY, Léa, SCHERLINGER, Marc, SCHLEINITZ, Nicolas, Sellam, Jérémie, Sentilhes, Loïc, SERVAIS, Aude, SMETS, Perrine, SORDET, Christelle, SOUBRIER, Martin, STANKOVIC-STOJANOVIC, Katia, URBANSKI, Geoffrey, VEIT, Véronique, WEBER, Emmanuelle, YELNIK, Cécile, de Frémont, Grégoire Martin, Costedoat-Chalumeau, Nathalie, Lazaro, Estibaliz, Belkhir, Rakiba, Guettrot-Imbert, Gaëlle, Morel, Nathalie, Nocturne, Gaétane, Molto, Anna, Goulenok, Tiphaine, Diot, Elisabeth, Perard, Laurent, Ferreira-Maldent, Nicole, Le Besnerais, Maelle, Limal, Nicolas, Martis, Nihal, Abisror, Noémie, Debouverie, Odile, Richez, Christophe, Sobanski, Vincent, Maurier, François, Sauvetre, Gaëtan, Levesque, Hervé, Timsit, Marie-Agnès, Tieulié, Nathalie, Orquevaux, Pauline, Bienvenu, Boris, Mahevas, Matthieu, Papo, Thomas, Lartigau-Roussin, Céline, Chauvet, Elodie, Berthoux, Emilie, Sarrot-Reynauld, Françoise, Raffray, Loïc, Couderc, Marion, Silva, Nicolas Martin, Jourde-Chiche, Noémie, Belhomme, Nicolas, Thomas, Thierry, Poindron, Vincent, Queyrel-Moranne, Viviane, Delforge, Juliette, Le Ray, Camille, Pannier, Emmanuelle, Mariette, Xavier, Le Guern, Véronique, and Seror, Raphaèle

Published
2023
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19. Dysregulation of neutrophil oxidant production and interleukin-1–related cytokines in granulomatosis with polyangiitis.

Author

Amsler, Jennifer, Everts-Graber, Judith, Martin, Katherine R, Roccabianca, Arnaud, Lopes, Chloé, Tourneur, Léa, Mocek, Julie, Karras, Alexandre, Naccache, Jean-Marc, Bonnotte, Bernard, Samson, Maxime, Hanslik, Thomas, Puéchal, Xavier, Terrier, Benjamin, Guillevin, Loïc, Néel, Antoine, Mouthon, Luc, and Witko-Sarsat, Véronique

Subjects
PROTEIN metabolism, RESEARCH funding, NEUTROPHILS, DISEASE remission, GRANULOMATOSIS with polyangiitis, REACTIVE oxygen species, ANTIGENS, LONGITUDINAL method, CYTOKINES, INFLAMMATION, INTERLEUKINS, GLUCOCORTICOIDS
Abstract

Objectives Neutrophils play a key role in ANCA-associated vasculitis, both as targets of autoimmunity and as facilitators of vascular damage. In granulomatosis with polyangiitis (GPA), the data regarding the production of reactive oxygen species (ROS) in neutrophils are unclear. Further, recent data suggests that ROS production could have an anti-inflammatory effect through the regulation of inflammasomes and IL-1–related cytokines. We aimed to analyse ROS production in neutrophils from patients with GPA and investigate its association with IL-1–related cytokines and the autoantigen PR3. Methods Seventy-two GPA patients with disease flare were included in the NEUTROVASC prospective cohort study. ROS production in whole blood of patients with active GPA was evaluated and compared with that in the same patients in remission or healthy controls. Associations between ROS production, PR3 membrane expression on neutrophils, serum levels of IL-1–related cytokines as well as inflammasome-related proteins were analysed. Results We observed a robust defect in ROS production by neutrophils from patients with active GPA compared with healthy controls, independent of glucocorticoid treatment. Serum levels of IL-1–related cytokines were significantly increased in GPA patients, particularly in patients with kidney involvement, and levels of these cytokines returned to normal after patients achieved remission. Further, inflammasome-related proteins were significantly dysregulated in the cytosol of neutrophils as well as the serum from GPA patients. Conclusion Our data suggests that ROS production and regulation of inflammasomes in neutrophils from patients with GPA are disturbed and may be a potential therapeutic target. Trial registration ClinicalTrials.gov, https://www.clinicaltrials.gov , NCT01862068. [ABSTRACT FROM AUTHOR]

Published
2024
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20. ANCA-associated vasculitides: Recommendations of the French Vasculitis Study Group on the use of immunosuppressants and biotherapies for remission induction and maintenance

Author

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

Published
2020
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22. Symptom-based stratification of patients with primary Sjögren's syndrome: multi-dimensional characterisation of international observational cohorts and reanalyses of randomised clinical trials

Author

Devauchelle-Pensec, Valerie, Dieude, Philippe, Dubost, Jean Jacques, Fauchais, Anne-Laure, Goeb, Vincent, Hachulla, Eric, Larroche, Claire, Le Guern, Véronique, Morel, Jacques, Perdriger, Aleth, Puéchal, Xavier, Rist, Stephanie, Sen, Damien, Sibilia, Jean, Vittecoq, Olivier, Benessiano, Joelle, Tubiana, Sarah, Inamo, Karine, Gaete, Stanie, Batouche, Djilali, Molinari, Domitille, Randrianandrasana, Mickael, Pane, Isabelle, Abbe, Adeline, Baron, Gabriel, Ravaud, Philippe, Gottenberg, Jacques-Eric, Saraux, Alain, Devauchelle-Pensec, Valérie, Hayem, Gilles, Hatron, Pierre, Sene, Damien, Zarnitsky, Charles, Furlan, Valérie, Perrodeau, Elodie, Seror, Raphaele, Mariette, Xavier, Brown, S, Navarro, N Coy, Pitzalis, C, Emery, P, Pavitt, S, Gray, J, Hulme, C, Hall, F, Busch, R, Smith, P, Dawson, L, Bombardieri, M, Ng, W-F, Pease, C, Price, E, Sutcliffe, N, Woods, C, Ruddock, S, Everett, C, Reynolds, C, Skinner, E, Poveda-Gallego, A, Rout, J, Macleod, I, Rauz, S, Bowman, S, Ng, Wan-Fai, Bowman, Simon J, Griffiths, Bridget, Hall, Frances, Bacaba, Elalaine C, Frankland, Helen, Moots, Robert, Chadravarty, Kuntal, Lamabadusuriya, Shamin, Bombardieri, Michele, Pitzalis, Constantino, Sutcliffe, Nurhan, Breston, Celia, Gendi, Nagui, Culfear, Karen, Riddell, Claire, Hamburger, John, Richards, Andrea, Rauz, Saaeh, Brailsford, Sue, Dasgin, Joanne, Logan, Joanne, Mulherin, Diarmuid, Andrews, Jacqueline, Emery, Pau, McManus, Alison, Pease, Colin, Pickles, David, Booth, Alison, Regan, Marian, Kin, Jon King, Holt, Amanda, Dimitroulas, Theodoros, Kadiki, Lucy, Kaur, Daljit, Kitas, George, Khan, Abdul, Cosier, Tracey, Panthakalam, Mintrim, Kell, Lloyd, Mark, Moore, Lisa, Gordon, Esther, Lawson, Cathy, Gupta, Monica, Hunter, John, Stirton, Lesley, Ortiz, Gill, Price, Elizabeth, Pelger, Suzannah, Gorman, Claire, Hans, Balinder, Clunie, Gavin, Lane, Suzanne, Rose, Ginny, Cuckow, Sue, Batley, Michael, Einosas, Ruby, Knight, Susan, Symmons, Deborah, Jones, Beverley, Carr, Andrew, Edgar, Suzanne, Figuereido, Francisco, Foggo, Heather, Lendrem, Dennis, Macleod, Iain, Mitchell, Sheryl, Downie, Christine, Tarn, Jessica, Locke, James, Al-Ali, Shereen, Legg, Sarah, Mirza, Kamran, Hargreaves, Ben, Hetherington, Laura, Jones, Adrian, Lanyon, Peter, Muir, Alice, White, Paula, Young-Min, Steven, Pugmire, Susan, Vadivelu, Saravanan, Cooper, Annie, Watkins, Marianne, Field, Anne, Kaye, Stephen, Mewar, Devesh, Medcalf, Patricia, Tomlinson, Pamela, Whiteside, Debbie, McHugh, Neil, Pauling, John, James, Julie, Dowden, Andrea, Akil, Mohammed, McDermott, Jayne, Godia, Olivia, Coady, David, Kidd, Elizabeth, Palmer, Lynne, Li, Charles, Bartrum, Sarah, Mead, De, Dasgupta, Bhaskar, Katsande, Victoria, Long, Pamela, Vermaak, Erin, Turner, Janet, Chandra, Usha, MacKay, Kirsten, Fedele, Stefano, Ferenkeh-Koroma, Ada, Giles, Ian, Isenberg, David, MaConnell, Helena, Ahwiren, Nyarko, Porter, Stephen, Allcoa, Paul, McLaren, John, Tarn, Jessica R, Howard-Tripp, Nadia, Lendrem, Dennis W, Skelton, Andrew J, James, Katherine, McMeekin, Peter, Hackett, Katie L, Lendrem, B Clare, Casement, John, Pease, Colin T, Emery, Paul, Pitzalis, Costantino, Saravanan, Vadivelu, Johnsen, Svein J A, Norheim, Katrine B, Omdal, Roald, Stocken, Deborah, Everett, Colin, Fernandez, Catherine, and Isaacs, John D

Published
2019
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25. Combining ts- and a bDMARD in refractory rheumatoid arthritis: an unusual adverse event.

Author

Sztejkowski, Cédric, Sibilia, Jean, Danion, François, Mertz, Philippe, Elodie, Fels, Kassegne, Loïc, Boyer, Pierre, Puéchal, Xavier, Gottenberg, Jacques-Eric, and Scherlinger, Marc

Subjects
RHEUMATOID arthritis diagnosis, LYMPHATIC disease diagnosis, BIOTHERAPY, COMBINATION drug therapy, BIOPSY, DRUG side effects, CHEST pain, IMMUNOSUPPRESSIVE agents, RHEUMATOID arthritis, COMPUTED tomography, ANTIRHEUMATIC agents, TREATMENT effectiveness, ANGIOGRAPHY, FEVER, PERSPIRATION, ANTITUBERCULAR agents, CO-trimoxazole, DYSPNEA, LYMPHATIC diseases, CEFTRIAXONE
Abstract

The article describes the case of a 52-year-old woman diagnosed with ACPA+/RF+ difficult-to-treat erosive rheumatoid arthritis (RA), which had been progressing since 1998. Topics discussed include observation on a synovial biopsy of the knee, results of combined therapy initiation, and a diagnosis of lymph node granulomatosis due to a Tropheryma whipplei infection in an immunocompromised patient.

Published
2024
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27. ANCA-associated scleritis: impact of ANCA on presentation, response to therapy and outcome.

Author

Perray, Laura, Nguyen, Yann, Refregiers, Gaëlle Clavel, Chazal, Thibaud, Héron, Emmanuel, Pouchelon, Clara, Dunogué, Bertrand, Costedoat-Chalumeau, Nathalie, Murarasu, Anne, Régent, Alexis, Puéchal, Xavier, Thoreau, Benjamin, Lifermann, François, Graveleau, Julie, Hié, Miguel, Froissart, Antoine, Baudet, Antoine, Deroux, Alban, Lavigne, Christian, and Puigrenier, Sébastien

Subjects
RESEARCH, C-reactive protein, GLUCOCORTICOIDS, RITUXIMAB, DISEASE progression, CONFIDENCE intervals, ANTINEUTROPHIL cytoplasmic antibodies, SCLERA, ACQUISITION of data, RETROSPECTIVE studies, CASE-control method, TERTIARY care, TREATMENT effectiveness, RISK assessment, MEDICAL records, DESCRIPTIVE statistics, EYE diseases, LONGITUDINAL method, SYMPTOMS
Abstract

Objectives To describe the characteristics, treatment and outcome of isolated ANCA-associated scleritis at diagnosis compared with idiopathic scleritis with negative ANCA tests. Methods This retrospective multicentre case–control study was performed within the French Vasculitis Study Group (FVSG) network and in three French tertiary ophthalmologic centres. Data from patients with scleritis without any systemic manifestation and with positive ANCA results were compared with those of a control group of patients with idiopathic scleritis with negative ANCA tests. Results A total of 120 patients, including 38 patients with ANCA-associated scleritis and 82 control patients, diagnosed between January 2007 and April 2022 were included. The median follow-up was 28 months (IQR 10–60). The median age at diagnosis was 48 years (IQR 33–60) and 75% were females. Scleromalacia was more frequent in ANCA-positive patients (P  = 0.027) and 54% had associated ophthalmologic manifestations, without significant differences. ANCA-associated scleritis more frequently required systemic medications, including glucocorticoids (76% vs 34%; P  < 0.001), and rituximab (P  = 0.03) and had a lower remission rate after the first- and second-line treatment. Systemic ANCA-associated vasculitis (AAV) occurred in 30.7% of patients with PR3- or MPO-ANCA, after a median interval of 30 months (IQR 16.3–44). Increased CRP >5 mg/l at diagnosis was the only significant risk factor of progression to systemic AAV [adjusted hazard ratio 5.85 (95% CI 1.10, 31.01), P  = 0.038]. Conclusion Isolated ANCA-associated scleritis is mostly anterior scleritis with a higher risk of scleromalacia than ANCA-negative idiopathic scleritis and is more often difficult to treat. One-third of patients with PR3- or MPO-ANCA scleritis progressed to systemic AAV. [ABSTRACT FROM AUTHOR]

Published
2024
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28. Characteristics and outcome of ANCA-associated vasculitides induced by anti-thyroid drugs: a multicenter retrospective case-control study

Author

Karadag, Omer, Culerrier, Julien, Nguyen, Yann, Luca, Luminita, Andre, Baptiste, Droumaguet, Celine, Ingen-Housz-Oro, Saskia, Duffau, Pierre, Marchand-Adam, Sylvain, Teixeira, Vitor, Regent, Alexis, Gauckler, Philipp, Jayne, David, Kronbichler, Andreas, Sadioglu Cagdas, Oznur, Yazici, Ayten, Celfe, Ayse, Yasar Bilge, Sule, Yildrim, TUBA, Ögüt, Tahir Saygin, Yazisiz, Veli, Bes, Cemal, Terrier, Benjamin, Puéchal, Xavier, Flossmann, Oliver, Lamprecht, Peter, Yacyshyn, Elaine, Pagnoux, Christian, Vandergheynst, Frédéric, Delvino, Paolo, Padoan, Roberto, Jego, Patrick, Poindron, Vincent, Milesi, Anne-Marie, Ruivard, Marc, Dernoncourt, Amandine, Servettaz, Amélie, Lebas, Celine, Aouba, Achille, and Lechtman, Sarah

Published
2023

31. Autoimmune and inflammatory diseases associated with chronic myelomonocytic leukemia: A series of 26 cases and literature review

Author

Grignano, Eric, Mekinian, Arsene, Braun, Thorsten, Liozon, Eric, Hamidou, Mohamed, Decaux, Olivier, Puéchal, Xavier, Kahn, Jean Emmanuel, Schoindre, Yoland, Rossignol, Julien, Lortholary, Olivier, Lioger, Bertrand, Hermine, Olivier, Park, Sophie, Ades, Lionel, Montestruc, François, Ricard, Laure, Gardin, Claude, Fenaux, Pierre, and Fain, Olivier

Published
2016
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32. Association Between Plasma Rituximab Concentration and the Risk of Major Relapse in Antineutrophil Cytoplasmic Antibody–Associated Vasculitides During Rituximab Maintenance Therapy.

Author

Khoudour, Nihel, Delestre, Florence, Jabot‐Hanin, Fabienne, Jouinot, Anne, Nectoux, Juliette, Letouneur, Franck, Izac, Brigitte, Vidal, Michel, Guillevin, Loïc, Puéchal, Xavier, Charles, Pierre, Terrier, Benjamin, and Blanchet, Benoit

Subjects
DNA analysis, RITUXIMAB, CONFIDENCE intervals, SINGLE nucleotide polymorphisms, HEALTH outcome assessment, ANTINEUTROPHIL cytoplasmic antibodies, REGRESSION analysis, DISEASE relapse, GENOTYPES, DESCRIPTIVE statistics, SURVIVAL analysis (Biometry), DRUG monitoring, LOGISTIC regression analysis, ODDS ratio, SENSITIVITY & specificity (Statistics), VASCULITIS, PHARMACODYNAMICS, DISEASE risk factors
Abstract

Objective: Interindividual variability in response to rituximab remains unexplored in antineutrophil cytoplasmic antibody (ANCA)–associated vasculitides. Rituximab pharmacokinetics (PK) and pharmacodynamics (PD) as well as genetic polymorphisms could contribute to variability. This ancillary study of the MAINRITSAN 2 trial aimed to explore the relationship between rituximab plasma concentration, genetic polymorphisms in PK/PD candidate genes, and clinical outcomes. Methods: Patients included in the MAINRITSAN2 trial (ClinicalTrials.gov identifier: NCT01731561) were randomized to receive a 500‐mg fixed‐schedule rituximab infusion or an individually tailored regimen. Rituximab plasma concentrations at month 3 (CM3) were assessed. DNA samples (n = 53) were genotyped for single‐nucleotide polymorphisms within 88 putative PK/PD candidate genes. The relationship between PK/PD outcomes and genetic variants was investigated using logistic linear regression in additive and recessive genetic models. Results: One hundred and thirty‐five patients were included. The frequency of underexposed patients (<4 μg/ml) in the fixed‐schedule group was statistically lower compared to that in the tailored‐infusion group (2.0% versus 18.0%; P = 0.02, respectively). Low rituximab plasma concentration at 3 months (CM3 <4 μg/ml) was an independent risk factor for major relapse (odds ratio 6.56 [95% confidence interval (95% CI) 1.26–34.09]; P = 0.025) at month 28 (M28). A sensitivity survival analysis also identified CM3 <4 μg/ml as an independent risk factor for major relapse (hazard ratio [HR] 4.81 [95% CI 1.56–14.82]; P = 0.006) and relapse (HR 2.70 [95% CI 1.02–7.15]; P = 0.046). STAT4 rs2278940 and PRKCA rs8076312 were significantly associated with CM3 but not with major relapse onset at M28. Conclusion: These results suggest that drug monitoring could be useful to individualize the schedule of rituximab administration within the maintenance phase. [ABSTRACT FROM AUTHOR]

Published
2023
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33. Clinico-radiological correlation and prognostic value of baseline chest computed tomography in eosinophilic granulomatosis with polyangiitis.

Author

Delestre, Florence, Brun, Anne-Laure, Thoreau, Benjamin, Taillé, Camille, Limal, Nicolas, Puéchal, Xavier, Mouthon, Luc, Guillevin, Loïc, Revel, Marie-Pierre, and Terrier, Benjamin

Subjects
LUNG disease diagnosis, THERAPEUTIC use of monoclonal antibodies, HOSPITALS, RESEARCH, CHEST X rays, PREDICTIVE tests, SCIENTIFIC observation, RETROSPECTIVE studies, TREATMENT effectiveness, CHURG-Strauss syndrome, DESCRIPTIVE statistics, COMPUTED tomography
Abstract

Objectives While chest high-resolution CT (HRCT) is correlated to severity and prognosis in asthma, it has not been studied in eosinophilic granulomatosis with polyangiitis (EGPA). Our objective is to study the prognostic value of baseline HRCT in EGPA patients. Methods Retrospective, multicentre observational study in three French hospitals, including EGPA patients with available chest HRCT before any systemic treatment. Two experienced radiologists blinded to clinical data evaluated HRCT images using semi-quantitative scoring. HRCT characteristics were correlated with clinical features and outcome. Results Among 46 patients, 38 (82.6%) had abnormal parenchymal findings on HRCT, including bronchial wall thickening (69.6%), mosaic perfusion (63.0%), ground-glass opacities (32.6%), bronchiectasis (30.4%), mucous plugging (21.7%) and consolidations (17.4%). Patients were clustered into three groups depending on HRCT features: ground-glass pattern, i.e. with ground-glass opacities with or without bronchial abnormalities (group 1, 28.3%), bronchial pattern (group 2, 41.3%) and extra-pulmonary pattern with no significant abnormality (group 3, 30.4%). Group 2 showed less frequent cardiac involvement (31.6 vs 46.2 and 42.9% in groups 1 and 3), more frequent positive ANCA (52.6 vs 0.0 and 14.3%) and higher eosinophil count (median 7510 vs 4000 and 4250/mm3). Group 1 showed worse prognosis with more frequent steroid-dependency (58.3 vs 11.1 and 28.6%) and requirement for mepolizumab (25.0 vs 11.1 and 7.1%). Conversely, group 2 showed a better outcome with higher rates of remission (88.9 vs 41.6 and 71.4%). Conclusion Chest HRCT at diagnosis of EGPA may have prognostic value and help clinicians better manage these patients. [ABSTRACT FROM AUTHOR]

Published
2023
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36. Identification of EPX variants in Human Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)

Author

David, Clémence, Hamel, Yamina, Smahi, Asma, Diot, Elisabeth, Benhamou, Ygal, Girszyn, Nicolas, Le Gallou, Thomas, Lifermann, François, Godmer, Pascal, Maurier, François, Cottin, Vincent, Grados, Aurélie, Aumaitre, Olivier, Néel, Antoine, Pugnet, Grégory, Masson, Cecile, Puéchal, Xavier, Mouthon, Luc, Guillevin, Loïc, Bienvenu, Thierry, and Terrier, Benjamin

Published
2023
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38. Biologic targeted therapies in pediatric rheumatology

Author

Guillaume-Czitrom, Séverine, Bachelez, Hervé, Berthelot, Jean-Marie, Bouhnik, Yoram, Bruckert, Eric, Claudepierre, Pascal, Constantin, Arnaud, de Bandt, Michel, Fautrel, Bruno, Gaudin, Philippe, Goëb, Vincent, Gossec, Laure, Gottenberg, Jacques-Eric, Goupille, Philippe, Hachulla, Eric, Huet, Isabelle, Jullien, Denis, Launay, Odile, Lemannf, Marc, Lequerré, Thierry, Maillefert, Jean-Francis, Mariette, Xavier, Marolleau, Jean-Pierre, Martinez, Valérie, Masson, Charles, Morel, Jacques, Mouthon, Luc, Pham, Thao, Pol, Stanislas, Puéchal, Xavier, Richette, Pascal, Saraux, Alain, Schaeverbeke, Thierry, Sellam, Jérémie, Blacher, Jacques, Soubrier, Martin, Sudre, Anne, Viguier, Manuelle, Vittecoq, Olivier, Wendling, Daniel, and Sibilia, Jean

Published
2014
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39. Reply

Author

Puéchal, Xavier, Pagnoux, Christian, and Guillevin, Loïc

Published
2018
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40. Recommendations for using TNFα antagonists and French Clinical Practice Guidelines endorsed by the French National Authority for Health

Author

Goëb, Vincent, Ardizzone, Marc, Arnaud, Laurent, Avouac, Jérôme, Baillet, Athan, Belot, Alexandre, Bouvard, Béatrice, Coquerelle, Pascal, Dadoun, Sabrina, Diguet, Alain, Launay, David, Lebouc, Danielle, Loulergue, Pierre, Mahy, Sophie, Mestat, Pascal, Mouterde, Gaël, Terrier, Benjamin, Varoquier, Coralie, Verdet, Mathieu, Puéchal, Xavier, and Sibilia, Jean

Published
2013
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41. ANCA-Associated Vasculitides Valvular Impairment: Multicenter Retrospective Study and Systematic Review of the Literature

Author

Jeantin, Lina, Lenfant, Tiphaine, Bataille, Pierre, de Boysson, Hubert, Cathébras, Pascal, Agard, Christian, Faguer, Stanislas, Poindron, Vincent, Ruivard, Marc, Silva, Nicolas Martin, Monge, Matthieu, Guillevin, Loic, Puéchal, Xavier, Terrier, Benjamin, Dechartres, Agnès, Charles, Pierre, Institut Mutualiste de Montsouris (IMM), Centre Hospitalier Boulogne-sur-mer, CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), CHU Saint-Etienne, Centre hospitalier universitaire de Nantes (CHU Nantes), Nantes Université (Nantes Univ), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), CHU Strasbourg, CHU Estaing [Clermont-Ferrand], CHU Clermont-Ferrand, Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Institut Pierre Louis d'Epidémiologie et de Santé Publique (iPLESP), Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), and Dechartres, Agnès

Subjects
[SDV] Life Sciences [q-bio], [SDV]Life Sciences [q-bio]
Abstract

International audience; Objective While myocardial impairment is a predictor of poor prognosis in antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV), little is known about valvular involvement. This study aims at describing the clinical presentation, management, and outcome of endocarditis associated with AAV. Methods We conducted a multicenter retrospective study in centers affiliated with the French Vasculitis Study Group. We included patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), or eosinophilic GPA with endocardial impairment. A systematic review was then performed through PubMed, Embase, and Cochrane Library from inception up to September 2020. Results The retrospective cohort included 9 patients (82%) with GPA, 1 (9%) with MPA, and 1 (9%) with unclassified AAV. Clinical presentation included acute valvular insufficiency (n = 7, 64%), cardiac failure (n = 3, 27%), dyspnea (n = 3, 27%), and no symptoms (n = 2, 18%). The aortic valve was the most frequently affected (n = 8/10, 80%), and vegetations were noted in 4 of 10 patients (40%). Six patients (55%) underwent surgical valvular replacement. No death from endocarditis was reported. The systematic review retrieved 42 patients from 40 references: 30 (71%) had GPA, 21 (50%) presented with vegetations, the aortic valve (n = 26, 62%) was the most frequently involved. Valvular replacement was required in 20 cases (48%) and 5 patients (13%) died from the endocarditic impairment. Conclusion Endocarditis is a rare and potentially life-threatening manifestation of AAV. Acute valvular insufficiency may lead to urgent surgery. Implementing transthoracic echocardiography in standard assessment at baseline and follow-up of AAV might reduce the delay to diagnosis and allow earlier immunosuppressive treatment before surgery is needed.

Published
2022
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44. Adding Azathioprine to Remission‐Induction Glucocorticoids for Eosinophilic Granulomatosis With Polyangiitis (Churg‐Strauss), Microscopic Polyangiitis, or Polyarteritis Nodosa Without Poor Prognosis Factors: A Randomized, Controlled Trial

Author

Puéchal, Xavier, Pagnoux, Christian, Baron, Gabriel, Quémeneur, Thomas, Néel, Antoine, Agard, Christian, Lifermann, François, Liozon, Eric, Ruivard, Marc, Godmer, Pascal, Limal, Nicolas, Mékinian, Arsène, Papo, Thomas, Ruppert, Anne‐Marie, Bourgarit, Anne, Bienvenu, Boris, Geffray, Loïck, Saraux, Jean‐Luc, Diot, Elisabeth, Crestani, Bruno, Delbrel, Xavier, Sailler, Laurent, Cohen, Pascal, Le Guern, Véronique, Terrier, Benjamin, Groh, Matthieu, Le Jeunne, Claire, Mouthon, Luc, Ravaud, Philippe, Guillevin, Loïc, Lavigne, C., Gille, T., Le Guenno, G., Rieu, V., André, M., Goulenok, T., Federici, L., Szmania, I., Bonnotte, B., Vinzio, S., Bezanahary, H., Ly, K. H., Khouatra, C., Lega, J. C., Dunand, J. F., Hamidou, M., Ponge, T., Brihaye, B., Chauveheid, P., Dawidowicz, K., Bussone, G., Crabol, Y., Fois, E., de Menthon, M., Szwebel, T. A., Arlet, J. B., Israel‐Biet, D., Bloch‐Queyrat, C., Bachmeyer, C., Lavole, A., Wislez, M., Soria, P., Viallard, J.‐F., Landron, C., Poindron, V., Pourrat, J., Magnant, J., Kyndt, X., and Meckenstock, R.

Published
2017
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45. Characteristics and Management of IgA Vasculitis (Henoch‐Schönlein) in Adults: Data From 260 Patients Included in a French Multicenter Retrospective Survey

Author

Audemard‐Verger, Alexandra, Terrier, Benjamin, Dechartres, Agnès, Chanal, Johan, Amoura, Zahir, Le Gouellec, Noémie, Cacoub, Patrice, Jourde‐Chiche, Noémie, Urbanski, Geoffrey, Augusto, Jean‐François, Moulis, Guillaume, Raffray, Loic, Deroux, Alban, Hummel, Aurélie, Lioger, Bertrand, Catroux, Mélanie, Faguer, Stanislas, Goutte, Julie, Martis, Nihal, Maurier, François, Rivière, Etienne, Sanges, Sébastien, Baldolli, Aurélie, Costedoat‐Chalumeau, Nathalie, Roriz, Mélanie, Puéchal, Xavier, André, Marc, Lavigne, Christian, Bienvenu, Boris, Mekinian, Arsène, Zagdoun, Elie, Girard, Charlotte, Bérezné, Alice, Guillevin, Loïc, Thervet, Eric, and Pillebout, Evangéline

Published
2017
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48. Polyangéite microscopique.

Author

Puéchal, Xavier

Subjects
*OVERALL survival, *GLUCOCORTICOIDS, *RITUXIMAB, *PROGNOSIS, *IMMUNOSUPPRESSIVE agents
Abstract

La polyangéite microscopique (PAM) est une vascularite nécrosante systémique touchant préférentiellement les vaisseaux de petit calibre. Une glomérulonéphrite et une hémorragie alvéolaire y sont fréquentes. Elle s'associe habituellement à des anticorps anti-cytoplasme des polynucléaires neutrophiles (ANCA) dirigés contre la myéloperoxidase (MPO), qui sont considérés comme pathogènes. La survie globale atteint maintenant 94 % à 5 ans mais des rechutes affectent entre un tiers et la moitié des patients. Le traitement d'induction permet d'obtenir une rémission complète dans ≥ 80 % des cas et est suivi d'un traitement d'entretien pour éviter une rechute. La base de la thérapeutique d'induction reste la corticothérapie, associée en première intention aux perfusions de cyclophosphamide ou de rituximab uniquement dans les formes sévères. En traitement d'entretien, le rituximab est plus efficace que les immunosuppresseurs conventionnels qu'il a supplantés. Pour les PAM de bon pronostic, une corticothérapie isolée suffit à contrôler la maladie sans rechute à 5 ans dans presque la moitié des cas. La négativation des ANCA en ELISA à 6 mois du traitement d'induction est associée à un moindre taux de rechute à 5 ans et rejoindra possiblement les objectifs thérapeutiques. Les échanges plasmatiques sont réalisés dans les formes sévères réfractaires à un traitement bien conduit ou en cas d'hémorragie alvéolaire massive. Les inhibiteurs du C5a représentent une nouvelle classe thérapeutique prometteuse, qui pourrait permettre de diminuer les doses de corticoïdes voire d'améliorer le pronostic rénal de cette vascularite touchant volontiers les sujets âgés, chez lesquels des protocoles de corticothérapie allégée devront aussi être évalués. Microscopic polyangiitis (MPA) is a necrotizing systemic vasculitis that preferentially affects small vessels. Glomerulonephritis and alveolar haemorrhage are common. MPA is usually associated with antineutrophil cytoplasmic antibodies (ANCA) directed againstmyeloperoxidase (MPO), which are considered pathogenic. Overall survival is 94% at 5 years, with relapses affecting between one third and one half of patients. Induction therapy achieves complete remission in ≥ 80% of cases and is followed by maintenance therapy to prevent relapses. The basis of induction treatment remains glucocorticoid therapy, combined only in severe forms with cyclophosphamide or rituximab infusions as first-line treatment. In maintenance treatment, rituximab is more effective than the traditional immunosuppressants that it has replaced. For MPA with a good prognosis, isolated glucocorticoid therapy is sufficient to control the disease without relapse at 5 years in almost half of the cases. Serologic remission (negative ANCA assay) within 6 months of induction treatment is associated with lower risk of relapse at 5 years and is likely to be incorporated into treatment goals in the future. Plasma exchange is performed in severe forms that are refractory to well-managed treatment or in cases of massive alveolar haemorrhage. C5a inhibitors represent a promising new therapeutic class, which could allow the reduction of glucocorticoid therapy while improving the renal prognosis of this vasculitis, which frequently affects elderly subjects in whom reduced glucocorticoid therapy protocols should also be evaluated. [ABSTRACT FROM AUTHOR]

Published
2023
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50. Abatacept therapy and safety management

Author

Pham, Thao, Bachelez, Hervé, Berthelot, Jean-Marie, Blacher, Jacques, Claudepierre, Pascal, Constantin, Arnaud, Fautrel, Bruno, Gaujoux-Viala, Cécile, Goëb, Vincent, Gossec, Laure, Goupille, Philippe, Guillaume-Czitrom, Séverine, Hachulla, Eric, Lequerré, Thierry, Marolleau, Jean-Pierre, Martinez, Valérie, Masson, Charles, Mouthon, Luc, Puéchal, Xavier, Richette, Pascal, Saraux, Alain, Schaeverbeke, Thierry, Soubrier, Martin, Viguier, Manuelle, Vittecoq, Olivier, Wendling, Daniel, Mariette, Xavier, and Sibilia, Jean

Published
2012
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