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3. Hypertension pulmonaire au cours de l’artérite de Takayasu

Author

Vismara, E., primary, Cid, M.C., additional, Aouba, A., additional, Jego, P., additional, Belhomme, N., additional, Emmerich, J., additional, Hanslik, T., additional, Haroche, J., additional, Puéchal, X., additional, Humbert, M., additional, Savale, L., additional, and Terrier, B., additional

Published
2024
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5. Atteinte glandulaires associées aux vascularites systémiques : une étude observationnelle

Author

Duhamel, N., primary, Le Gallou, T., additional, Chezel, J., additional, Deroux, A., additional, Roussin, C., additional, Keraen, J., additional, De Moreuil, C., additional, Jarrot, P.A., additional, Gerfaud-Valentin, M., additional, Maurier, F., additional, Melki, I., additional, Melboucy Belhkir, S., additional, Puéchal, X., additional, Terrier, B., additional, and Martis, N., additional

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

Author

Culerrier, J, Nguyen, Y, Karadag, O, Yasar Bilge, S, Demirci Yıldırım, T, Saygin Ögüt, T, Yazisiz, V, Bes, C, Celfe, A, Yazici, A, Sadioglu Cagdas, O, Kronbichler, A, Jayne, D, Gauckler, P, Regent, A, Teixeira, V, Marchand-Adam, S, Duffau, P, Ingen-Housz-Oro, S, Droumaguet, C, Andre, B, Luca, L, Lechtman, S, Aouba, A, Lebas, C, Servettaz, A, Dernoncourt, A, Ruivard, M, Milesi, A, Poindron, V, Jego, P, Padoan, R, Delvino, P, Vandergheynst, F, Pagnoux, C, Yacyshyn, E, Lamprecht, P, Flossmann, O, Puéchal, X, Terrier, B, Julien Culerrier, Yann Nguyen, Omer Karadag, Sule Yasar Bilge, Tuba Demirci Yıldırım, Tahir Saygin Ögüt, Veli Yazisiz, Cemal Bes, Ayse Celfe, Ayten Yazici, Oznur Sadioglu Cagdas, Andreas Kronbichler, David Jayne, Philipp Gauckler, Alexis Regent, Vitor Teixeira, Sylvain Marchand-Adam, Pierre Duffau, Saskia Ingen-Housz-Oro, Celine Droumaguet, Baptiste Andre, Luminita Luca, Sarah Lechtman, Achille Aouba, Celine Lebas, Amélie Servettaz, Amandine Dernoncourt, Marc Ruivard, Anne-Marie Milesi, Vincent Poindron, Patrick Jego, Roberto Padoan, Paolo Delvino, Frédéric Vandergheynst, christian pagnoux, Elaine Yacyshyn, Peter Lamprecht, Oliver Flossmann, Xavier Puéchal, Benjamin Terrier, Culerrier, J, Nguyen, Y, Karadag, O, Yasar Bilge, S, Demirci Yıldırım, T, Saygin Ögüt, T, Yazisiz, V, Bes, C, Celfe, A, Yazici, A, Sadioglu Cagdas, O, Kronbichler, A, Jayne, D, Gauckler, P, Regent, A, Teixeira, V, Marchand-Adam, S, Duffau, P, Ingen-Housz-Oro, S, Droumaguet, C, Andre, B, Luca, L, Lechtman, S, Aouba, A, Lebas, C, Servettaz, A, Dernoncourt, A, Ruivard, M, Milesi, A, Poindron, V, Jego, P, Padoan, R, Delvino, P, Vandergheynst, F, Pagnoux, C, Yacyshyn, E, Lamprecht, P, Flossmann, O, Puéchal, X, Terrier, B, Julien Culerrier, Yann Nguyen, Omer Karadag, Sule Yasar Bilge, Tuba Demirci Yıldırım, Tahir Saygin Ögüt, Veli Yazisiz, Cemal Bes, Ayse Celfe, Ayten Yazici, Oznur Sadioglu Cagdas, Andreas Kronbichler, David Jayne, Philipp Gauckler, Alexis Regent, Vitor Teixeira, Sylvain Marchand-Adam, Pierre Duffau, Saskia Ingen-Housz-Oro, Celine Droumaguet, Baptiste Andre, Luminita Luca, Sarah Lechtman, Achille Aouba, Celine Lebas, Amélie Servettaz, Amandine Dernoncourt, Marc Ruivard, Anne-Marie Milesi, Vincent Poindron, Patrick Jego, Roberto Padoan, Paolo Delvino, Frédéric Vandergheynst, christian pagnoux, Elaine Yacyshyn, Peter Lamprecht, Oliver Flossmann, Xavier Puéchal, and Benjamin Terrier

Abstract

Objective: Data on ANCA-associated vasculitis (AAV) induced by anti-thyroid drugs (ATD) are scarce. We aimed to describe the characteristics and outcome of these patients in comparison to primary AAV. Methods: We performed a retrospective multicentre study including patients with ATD-induced AAV. We focused on ATD-induced microscopic polyangiitis (MPA) and compared them with primary MPA by matching each case with four controls by gender and year of diagnosis. Results: Forty-five patients with ATD-induced AAV of whom 24 MPA were included. ANCA were positive in 44 patients (98%), including myeloperoxidase (MPO)-ANCA in 21 (47%), proteinase 3 (PR3)-ANCA in six (13%), and double positive MPO- and PR3-ANCA in 15 (33%). Main clinical manifestations were skin involvement (64%), arthralgia (51%) and glomerulonephritis (20%). ATD was discontinued in 98% of cases, allowing vasculitis remission in seven (16%). All the remaining patients achieved remission after glucocorticoids, in combination with rituximab in 11 (30%) or cyclophosphamide in four (11%). ATD were reintroduced in seven cases (16%) without any subsequent relapse. Compared with 96 matched primary MPA, ATD-induced MPA were younger at diagnosis (48 vs 65 years, P < 0.001), had more frequent cutaneous involvement (54 vs 25%, P 1⁄4 0.007), but less frequent kidney (38 vs 73%, P 1⁄4 0.02), and a lower risk of relapse (adjusted HR 0.07; 95% CI 0.01, 0.65, P 1⁄4 0.019). Conclusion: ATD-induced AAV were mainly MPA with MPO-ANCA, but double MPO- and PR3-ANCA positivity was frequent. The most common manifestations were skin and musculoskeletal manifestations. ATD-induced MPA were less severe and showed a lower risk of relapse than primary MPA.

Published
2024

9. Caractéristiques initiales et évolutives des périartérites noueuses associées au syndrome VEXAS et aux leucémies myélo-monocytaires chroniques comparativement aux formes primitives

Author

Cioni, P., primary, Nguyen, Y., additional, Rohmer, J., additional, Dupuy, H., additional, Puéchal, X., additional, Lazaro, E., additional, Pugnet, G., additional, Durel, C.A., additional, Conticini, E., additional, Lifermann, F., additional, Bielefeld, P., additional, Duffau, P., additional, Mahrhofer, H., additional, Régent, A., additional, Guillevin, L., additional, Trefond, L., additional, Agard, C., additional, Allain, J.S., additional, Berezne, A., additional, Mekinian, A., additional, Lacombe, V., additional, and Terrier, B., additional

Published
2023
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10. Atteinte des vaisseaux de gros calibre au cours des vascularites à ANCA : une étude multicentrique cas-témoin

Author

Monghal, V., primary, Puéchal, X., additional, Smets, P., additional, Vandergheynst, F., additional, Michel, M., additional, Diot, E., additional, Ramdani, Y., additional, Moulinet, T., additional, Dhôte, R., additional, Hautcoeur, A., additional, Lelubre, C., additional, Dominique, S., additional, Lebourg, L., additional, Melboucy Belhkir, S., additional, Wauters, N., additional, Carlotti, A., additional, Cachin, F., additional, Ebbo, M., additional, Jourde-Chiche, N., additional, Ludici, M., additional, Aumaître, O., additional, Andre, M., additional, Terrier, B., additional, and Trefond, L., additional

Published
2023
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11. Essai randomisé, contrôle et en double aveugle sur l’impact de la rosuvastatine sur les marqueurs d’athérosclérose infra-clinique chez les patients atteints de vascularite associée aux ANCA

Author

Terrier, B., primary, Pugnet, G., additional, Sirieix, M.E., additional, Quéméneur, T., additional, Puéchal, X., additional, Maurier, F., additional, Néel, A., additional, Benhamou, Y., additional, Bonnotte, B., additional, Schmidt, J., additional, Michon, A., additional, Baudet, A., additional, Charles, P., additional, Cohen-Aubart, F., additional, De Moreuil, C., additional, Dernis, E., additional, Belenotti, P., additional, Ruivard, M., additional, Aumaître, O., additional, Fain, O., additional, Seror, R., additional, Durel, C.A., additional, Jourde-Chiche, N., additional, Lazaro, E., additional, Armengol, G., additional, Bellien, J., additional, Ravaud, P., additional, Baron, G., additional, and Guillevin, L., additional

Published
2023
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12. Efficacité et tolérance d’une association de traitements ciblés indiqués pour l’asthme et pour les rhumatismes inflammatoires chroniques

Author

Etronnier, L., primary, Bourdin, A., additional, Lukas, C., additional, Gérard, B., additional, Constantin, A., additional, Berthoud, O., additional, Daien, C., additional, Flachaire, B., additional, Milin, M., additional, Puéchal, X., additional, Rempenault, C., additional, Soubrier, M., additional, and Morel, J., additional

Published
2023
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16. Remission and Low Disease Activity in Granulomatosis With Polyangiitis and Microscopic Polyangiitis: Prevalence and Impact on Damage Accrual

Author

Delvino, P, Sardanelli, F, Monti, S, Cohen, P, Puéchal, X, Mouthon, L, Montecucco, C, Guillevin, L, Terrier, B, Paolo Delvino, Federica Sardanelli, Sara Monti, Pascal Cohen, Xavier Puéchal, Luc Mouthon, Carlomaurizio Montecucco, Loïc Guillevin, Benjamin Terrier, Delvino, P, Sardanelli, F, Monti, S, Cohen, P, Puéchal, X, Mouthon, L, Montecucco, C, Guillevin, L, Terrier, B, Paolo Delvino, Federica Sardanelli, Sara Monti, Pascal Cohen, Xavier Puéchal, Luc Mouthon, Carlomaurizio Montecucco, Loïc Guillevin, and Benjamin Terrier

Abstract

Objective: To assess the prevalence and impact on damage accrual of different levels of disease activity in patients with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). Methods: Patients with GPA and MPA followed for ≥5 years in 2 different centers were included. Disease activity and damage were assessed using the Birmingham Vasculitis Activity Score (BVAS) and Vasculitis Damage Index (VDI), respectively. Three levels of remission were defined: complete remission (BVAS = 0, negative for antineutrophil cytoplasmic antibody [ANCA], off treatment), clinical remission off therapy (CROffT; BVAS = 0, positive for ANCA), and clinical remission on therapy (CROnT; BVAS = 0, negative or positive for ANCA, glucocorticoids ≤5 mg/day and/or immunosuppressant). A low disease activity state (LDAS) was defined as 0 < BVAS ≤3, low-dose glucocorticoids (≤7.5 mg/day), and/or immunosuppressant. Remission or LDAS were defined as prolonged when lasting ≥2 consecutive years. Results: A total of 167 patients were included: 128 (76.6%) with GPA, 39 (23.4%) with MPA, mean ± SD age 51.0 ± 16.7 years. During a 5-year follow-up, 10 patients (6.0%) achieved prolonged complete remission, 6 (3.6%) prolonged CROffT, 89 (53.3%) prolonged CROnT, 42 (25.1%) prolonged LDAS, and 20 (12.0%) never achieved LDAS. The VDI score at 5 years progressively worsened according to increasing levels of disease activity targets (complete remission, CROffT, CROnT, and LDAS). The mean ± SD 5-year VDI score was higher in patients not achieving prolonged remission compared to those who did (3.7 ± 2.0 versus 2.2 ± 1.9; P < 0.0001). By multivariate analysis, baseline ear, nose, and throat (P = 0.006), and lung involvement (P = 0.047) were negative predictors of prolonged remission. Conclusion: More than 60% of patients with GPA/MPA achieved prolonged remission, which was associated with better long-term outcomes. In contrast, prolonged LDAS correlated with increased damage accrual and was

Published
2023

17. Caractéristiques et évolution des vascularites associées aux ANCA induites par les antithyroïdiens de synthèse comparativement aux formes primitives : étude rétrospective multicentrique

Author

Culerrier, J., primary, Nguyen, Y., additional, Karadag, O., additional, Yasar Bilge, S., additional, Kronbichler, A., additional, Jayne, D., additional, Régent, A., additional, Teixeira, V., additional, Marchand-Adam, S., additional, Duffau, P., additional, Oro, S., additional, Droumaguet, C., additional, Andre, B., additional, Luca, L., additional, Lechtman, S., additional, Aouba, A., additional, Lebas, C., additional, Servettaz, A., additional, Puéchal, X., additional, and Terrier, B., additional

Published
2022
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19. Rituximab versus cyclophosphamide en traitement d’induction de la granulomatose avec polyangéite : essai thérapeutique émulé

Author

Puéchal, X., primary, Iudici, M., additional, Perrodeau, E., additional, Bonnotte, B., additional, Lifermann, F., additional, Le Gallou, T., additional, Karras, A., additional, Blanchard-Delaunay, C., additional, Quéméneur, T., additional, Aouba, A., additional, Aumaître, O., additional, Cottin, V., additional, Hamidou, M., additional, Ruivard, M., additional, Cohen, P., additional, Mouthon, L., additional, Guillevin, L., additional, Ravaud, P., additional, Porcher, R., additional, and Terrier, B., additional

Published
2022
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20. Évaluation de la pirfénidone chez les patients présentant une fibrose pulmonaire associée aux anticorps anti-myéloperoxydase (MPO) ou à une vascularite associée aux anti-MPO : résultats de l’essai prospectif PIRFENIVAS

Author

London, J., primary, Chassagnon, G., additional, Puéchal, X., additional, Régent, A., additional, Legendre, P., additional, Cohen, P., additional, Revel, M.P., additional, Lefevre, E., additional, Borie, R., additional, Jilet, L., additional, Abdoul, H., additional, and Terrier, B., additional

Published
2022
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21. Sclérites et épisclérites associées aux anticorps anti-cytoplasme des polynucléaires neutrophiles : présentation initiale et pronostic

Author

Perray, L., primary, Nguyen, Y., additional, Clavel-Refregiers, G., additional, Chazal, T., additional, Héron, E., additional, Puéchal, X., additional, Pouchelon, C., additional, Thoreau, B., additional, Régent, A., additional, Murarasu, A., additional, Dunogué, B., additional, Costedoat-Chalumeau, N., additional, Lifermann, F., additional, Deroux, A., additional, Graveleau, J., additional, Vasco, C., additional, Hié, M., additional, Froissart, A., additional, Brézin, A., additional, and Terrier, B., additional

Published
2022
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22. Efficacité à long terme des schémas d’induction de la rémission au cours de la granulomatose éosinophilique avec polyangéite : résultats de l’essai REOVAS

Author

Dutertre, M., primary, Pugnet, G., additional, De Moreuil, C., additional, Bonnotte, B., additional, Benhamou, Y., additional, Chauveau, D., additional, Diot, E., additional, Duffau, P., additional, Limal, N., additional, Néel, A., additional, Urbansky, G., additional, Jourde-Chiche, N., additional, Fauchais, A.L., additional, Dossier, A., additional, Schleinitz, N., additional, Jilet, L., additional, Guillevin, L., additional, Abdoul, H., additional, Puéchal, X., additional, and Terrier, B., additional

Published
2022
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23. Étude des granulomatoses avec polyangéites réfractaires au traitement d’induction

Author

Sorin, B., primary, Iudici, M., additional, Guerry, M.J., additional, Samson, M., additional, Bielefeld, P., additional, Maillet, T., additional, Nouvier, M., additional, Karras, A., additional, Christian, L., additional, Durel, C.A., additional, Fabre, M., additional, Charles, P., additional, Lanteri, A., additional, Pugnet, G., additional, Riviere, F., additional, Le Gueno, G., additional, Guillevin, L., additional, Puéchal, X., additional, and Terrier, B., additional

Published
2022
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24. Performance de modèles de prédiction du risque de rechute et d’infection sévère à la fin du traitement d’entretien par rituximab à 18 mois au cours des vascularites à ANCA

Author

Delestre, F., primary, Charles, P., additional, Samson, M., additional, Néel, A., additional, Faguer, S., additional, Karras, A., additional, Lifermann, F., additional, Godmer, P., additional, Hanrotel-Saliou, C., additional, Martin-Silva, N., additional, Pugnet, G., additional, Maurier, F., additional, Le Gallou, T., additional, Quéméneur, T., additional, Méaux-Ruault, N., additional, Viallard, J.F., additional, Puéchal, X., additional, Guillevin, L., additional, Porcher, R., additional, and Terrier, B., additional

Published
2022
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25. Fardeau lié aux traitements et adhésion aux traitements immunosuppresseurs chez les patients en médecine interne : une étude observationnelle

Author

Mettler, C., primary, Le Guern, V., additional, Legendre, P., additional, Morbieu, C., additional, Szwebel, T.A., additional, Puéchal, X., additional, Cohen, P., additional, Kardaoui, H., additional, Moores, A., additional, Mouthon, L., additional, Nathalie, C.C., additional, and Terrier, B., additional

Published
2022
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26. Atteintes valvulaires cardiaques des vascularites à ANCA : étude multicentrique rétrospective et revue systématique de la littérature

Author

Jeantin, L., primary, Lenfant, T., additional, Bataille, P., additional, De Boysson, H., additional, Cathébras, P., additional, Agard, C., additional, Faguer, S., additional, Deschartres, A., additional, Poindron, V., additional, Ruivard, M., additional, Martin-Silva, N., additional, Monge, M., additional, Guillevin, L., additional, Puéchal, X., additional, Terrier, B., additional, and Charles, P., additional

Published
2022
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29. OP0066 IMPACT OF DIAGNOSIS AND TREATMENT OF TROPHERYMA WHIPPLEI INFECTION IN PATIENTS WITH PRE-EXISTING CHRONIC INFLAMMATORY RHEUMATIC DISEASES: DATA FROM THE NATIONAL Tw-IRD REGISTRY

Author

Damien, C. P., primary, Puéchal, X., additional, Degboe, Y., additional, Kostine, M., additional, Michaut, A., additional, Ramon, A., additional, Wendling, D., additional, Costedoat-Chalumeau, N., additional, Richette, P., additional, Marotte, H., additional, Vix, J., additional, Dubost, J. J., additional, Ottaviani, S., additional, Mouterde, G., additional, Grasland, A., additional, Frazier-Mironer, A., additional, Germain, V., additional, Coury-Lucas, F., additional, Tournadre, A., additional, Soubrier, M., additional, Brevet, P., additional, Cavalie, L., additional, Arnaud, L., additional, Richez, C., additional, Ruyssen-Witrand, A., additional, and Constantin, A., additional

Published
2022
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31. Rituximab en traitement d’entretien des vascularites à ANCA: analyse poolée et suivi à long terme des 277 patients suivis dans les essais prospectifs randomisés contrôlés MAINRITSAN

Author

Delestre, F., primary, Charles, P., additional, Porcher, R., additional, Samson, M., additional, Néel, A., additional, Faguer, S., additional, Karras, A., additional, Lifermann, F., additional, Godmer, P., additional, Hanrotel-Saliou, C., additional, Martin-Silva, N., additional, Pugnet, G., additional, Maurier, F., additional, Le Gallou, T., additional, Méaux-Ruault, N., additional, Viallard, J.F., additional, Hachulla, E., additional, Puéchal, X., additional, Guillevin, L., additional, and Terrier, B., additional

Published
2021
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33. Impact du traitement d’une infection à Tropheryma whipplei chez des patients atteints d’un rhumatisme inflammatoire chronique traités par DMARD : données du registre national WHIRIC

Author

Caillet Portillo, D., primary, Puéchal, X., additional, Degboe, Y., additional, Dubost, J.J., additional, Kostine, M., additional, Michaut, A., additional, Ramon, A., additional, Mouterde, G., additional, Vix, J., additional, Brevet, P., additional, Coury, F., additional, Costedoat-Chalumeau, N., additional, Frazier-Mironer, A., additional, Germain, V., additional, Grasland, A., additional, Marotte, H., additional, Ottaviani, S., additional, Richette, P., additional, Soubrier, M., additional, Tournadre, A., additional, Wendling, D., additional, Cavalie, L., additional, Arnaud, L., additional, Richez, C., additional, Ruyssen-Witrand, A., additional, and Constantin, A., additional

Published
2021
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34. Use of Biologics to Treat Relapsing and/or Refractory Eosinophilic Granulomatosis With Polyangiitis: Data From a European Collaborative Study

Author

Canzian A, Venhoff N, Urban ML, Sartorelli S, Ruppert AM, Groh M, Girszyn N, Taillé C, Maurier F, Cottin V, de Moreuil C, Germain V, Samson M, Jachiet M, Denis L, Rieu V, Smets P, Pugnet G, Deroux A, Durel CA, Aouba A, Cathébras P, Deligny C, Faguer S, Gil H, Godeau B, Lifermann F, Phin-Huynh S, Ruivard M, Bonniaud P, Puéchal X, Kahn JE, Thiel J, Dagna L, Guillevin L, Vaglio A, Emmi G, Terrier B, French Vasculitis Study Group (FVSG), the European EGPA Study Group., Canzian, A, Venhoff, N, Urban, Ml, Sartorelli, S, Ruppert, Am, Groh, M, Girszyn, N, Taillé, C, Maurier, F, Cottin, V, de Moreuil, C, Germain, V, Samson, M, Jachiet, M, Denis, L, Rieu, V, Smets, P, Pugnet, G, Deroux, A, Durel, Ca, Aouba, A, Cathébras, P, Deligny, C, Faguer, S, Gil, H, Godeau, B, Lifermann, F, Phin-Huynh, S, Ruivard, M, Bonniaud, P, Puéchal, X, Kahn, Je, Thiel, J, Dagna, L, Guillevin, L, Vaglio, A, Emmi, G, Terrier, B, and French Vasculitis Study Group (FVSG), the European EGPA Study Group.

Subjects
Adult, Male, medicine.medical_specialty, Immunology, Birmingham Vasculitis Activity Score, Omalizumab, Churg-Strauss Syndrome, Antibodies, Monoclonal, Humanized, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Interquartile range, Recurrence, Internal medicine, medicine, Immunology and Allergy, Humans, Immunologic Factors, 030212 general & internal medicine, Treatment Failure, Adverse effect, Glucocorticoids, Aged, Retrospective Studies, 030203 arthritis & rheumatology, Biological Products, business.industry, Middle Aged, medicine.disease, Asthma, Treatment Outcome, Rituximab, Female, Vasculitis, business, Granulomatosis with polyangiitis, Mepolizumab, medicine.drug
Abstract

OBJECTIVE To describe the efficacy and safety of biologics for the treatment of eosinophilic granulomatosis with polyangiitis (EGPA). METHODS A retrospective European collaborative study was conducted in patients with EGPA who received treatment with biologics for refractory and/or relapsing disease. RESULTS Among the 147 patients with EGPA included in the study, 63 received rituximab (RTX), 51 received mepolizumab (MEPO), and 33 received omalizumab (OMA). At the time of inclusion, the median Birmingham Vasculitis Activity Score (BVAS) was 8.5 (interquartile range [IQR] 5-13) in the RTX group, while the median BVAS in the OMA group was 2 (IQR 1-4.5) and the median BVAS in the MEPO group was 2 (IQR 1-5). In patients receiving RTX, the median BVAS declined both at 6 months (median 1, IQR 0-4.5) and at 12 months (median 0, IQR 0-2), and the frequency of remission, partial response, treatment failure, and stopping treatment due to adverse events was 49%, 24%, 24%, and 3%, respectively. For the treatment of glucocorticoid (GC)-dependent asthma, patients who received MEPO had a much better GC-sparing effect and overall response than did patients who received OMA. The frequency of remission, partial response, treatment failure, and stopping treatment due to adverse events was 15%, 33%, 48%, and 4%, respectively, in the OMA group and 78%, 10%, 8%, and 4%, respectively, in the MEPO group. Remission rates at 12 months were 76% and 82% among patients receiving MEPO at a doses of 100 mg and 300 mg, respectively. CONCLUSION These results suggest that RTX could be effective in treating relapses of EGPA vasculitis. MEPO is highly effective with a good safety profile in patients with GC-dependent asthma. Our data suggest that 100 mg MEPO monthly could be an acceptable dosage for first-line therapy in selected instances of EGPA, recognizing, however, that this has not been compared to the validated dosage of 300 mg monthly.

Published
2020

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

Author

Mettler, C., primary, Durel, CA., additional, Guilpain, P., additional, Bonnotte, B., additional, Cohen-Aubart, F., additional, Hamidou, M., additional, Lega, JC., additional, Guern, V. Le, additional, Lifermann, F., additional, Poindron, V., additional, Pugnet, G., additional, Servettaz, A., additional, Puéchal, X., additional, Guillevin, L., additional, and Terrier, L., additional

Published
2021
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38. Renal and overall outcomes of double-positive (ANCA and anti-GBM antibodies) patients compared to ANCA-associated vasculitis patients with severe renal involvement: A multicenter retrospective study with systematic renal pathology analysis

Author

Clerte, M, primary, Philip, R, additional, Levi, C, additional, Cornec-Le Gall, E, additional, Audard, V, additional, Huart, A, additional, Puéchal, X, additional, Touzot, M, additional, Rabot, N, additional, Thervet, É, additional, Aouba, A, additional, and Karras, A, additional

Published
2021
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39. Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Author

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, Terrier, B, Papo, Matthias, Sinico, Renato A, Teixeira, Vítor, Venhoff, Nils, Urban, Maria-Letizia, Iudici, Michele, Mahrhold, Juliane, Locatelli, Francesco, Cassone, Giulia, Schiavon, Franco, Seeliger, Benjamin, Neumann, Thomas, 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, Terrier, Benjamin, 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, Terrier, B, Papo, Matthias, Sinico, Renato A, Teixeira, Vítor, Venhoff, Nils, Urban, Maria-Letizia, Iudici, Michele, Mahrhold, Juliane, Locatelli, Francesco, Cassone, Giulia, Schiavon, Franco, Seeliger, Benjamin, Neumann, Thomas, 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, and Terrier, Benjamin

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 < 0.0001), more frequently had cutaneous manifestations (63% vs 38% and 34%, P = 0.03) and pulmonary nodules (25% vs 10% and 8%, P = 0.046), and lower median eosinophil count (1450 vs 5400 and 3224/mm3, P < 0.0001). Vasculitis relapse-free survival was shorter for PR3-ANCA (hazard ratio 6.05, P = 0.005) and MPO-ANCA patients (hazard ratio 1.88, P = 0.0002) compared with ANCA-negative patients. 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
2021

40. Renal involvement in eosinophilic granulomatosis with polyangiitis (EGPA): a multicentric retrospective study of 63 biopsy-proven cases

Author

Durel, C, Sinico, R, Teixeira, V, Jayne, D, Belenfant, X, Marchand-Adam, S, Pugnet, G, Gaultier, J, Le Gallou, T, Titeca-Beauport, D, Agard, C, Barbet, C, Bardy, A, Blockmans, D, Boffa, J, Bouet, J, Cottin, V, Crabol, Y, Deligny, C, Essig, M, Godmer, P, Guilpain, P, Hirschi-Santelmo, S, Rafat, C, Puéchal, X, Taillé, C, Karras, A, Durel, Cécile-Audrey, Sinico, Renato A, Teixeira, Vitor, Jayne, David, Belenfant, Xavier, Marchand-Adam, Sylvain, Pugnet, Gregory, Gaultier, Jacques, Le Gallou, Thomas, Titeca-Beauport, Dimitri, Agard, Christian, Barbet, Christelle, Bardy, Antoine, Blockmans, Daniel, Boffa, Jean-Jacques, Bouet, Julien, Cottin, Vincent, Crabol, Yoann, Deligny, Christophe, Essig, Marie, Godmer, Pascal, Guilpain, Philippe, Hirschi-Santelmo, Sandrine, Rafat, Cédric, Puéchal, Xavier, Taillé, Camille, Karras, Alexandre, Durel, C, Sinico, R, Teixeira, V, Jayne, D, Belenfant, X, Marchand-Adam, S, Pugnet, G, Gaultier, J, Le Gallou, T, Titeca-Beauport, D, Agard, C, Barbet, C, Bardy, A, Blockmans, D, Boffa, J, Bouet, J, Cottin, V, Crabol, Y, Deligny, C, Essig, M, Godmer, P, Guilpain, P, Hirschi-Santelmo, S, Rafat, C, Puéchal, X, Taillé, C, Karras, A, Durel, Cécile-Audrey, Sinico, Renato A, Teixeira, Vitor, Jayne, David, Belenfant, Xavier, Marchand-Adam, Sylvain, Pugnet, Gregory, Gaultier, Jacques, Le Gallou, Thomas, Titeca-Beauport, Dimitri, Agard, Christian, Barbet, Christelle, Bardy, Antoine, Blockmans, Daniel, Boffa, Jean-Jacques, Bouet, Julien, Cottin, Vincent, Crabol, Yoann, Deligny, Christophe, Essig, Marie, Godmer, Pascal, Guilpain, Philippe, Hirschi-Santelmo, Sandrine, Rafat, Cédric, Puéchal, Xavier, Taillé, Camille, and Karras, Alexandre

Abstract

Objective: Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small-vessel vasculitis characterized by asthma, hypereosinophilia and ANCA positivity in 40% of patients. Renal involvement is rare and poorly described, leading to this renal biopsy-proven based study in a large EGPA cohort. Methods: We conducted a retrospective multicentre study including patients fulfilling the 1990 ACR criteria and/or the 2012 revised Chapel Hill Consensus Conference criteria for EGPA and/or the modified criteria of the MIRRA trial, with biopsy-proven nephropathy. Results: Sixty-three patients [27 women, median age 60 years (18-83)] were included. Renal disease was present at vasculitis diagnosis in 54 patients (86%). ANCA were positive in 53 cases (84%) with anti-MPO specificity in 44 (83%). All patients had late-onset asthma. Peripheral neuropathy was present in 29 cases (46%), alveolar haemorrhage in 10 (16%). The most common renal presentation was acute renal failure (75%). Renal biopsy revealed pauci-immune necrotizing GN in 49 cases (78%). Membranous nephropathy (10%) and membranoproliferative GN (3%) were mostly observed in ANCA-negative patients. Pure acute interstitial nephritis was found in six cases (10%); important interstitial inflammation was observed in 28 (44%). All patients received steroids with adjunctive immunosuppression in 54 cases (86%). After a median follow-up of 51 months (1-296), 58 patients (92%) were alive, nine (14%) were on chronic dialysis and two (3%) had undergone kidney transplantation. Conclusion: Necrotizing pauci-immune GN is the most common renal presentation in ANCA-positive EGPA. ANCA-negative patients had frequent atypical renal presentation with other glomerulopathies such as membranous nephropathy. An important eosinophilic interstitial infiltration was observed in almost 50% of cases.

Published
2021

41. POS0117 RENAL AND OVERALL OUTCOMES OF DOUBLE-POSITIVE (ANCA AND ANTI-GBM ANTIBODIES) PATIENTS COMPARED TO ANCA-ASSOCIATED VASCULITIS PATIENTS WITH SEVERE RENAL INVOLVEMENT: A MULTICENTER RETROSPECTIVE STUDY WITH SYSTEMATIC RENAL PATHOLOGY ANALYSIS

Author

Clerte, M., primary, Philip, R., additional, Levi, C., additional, Cornec-Legall, E., additional, Audard, V., additional, Huart, A., additional, Puéchal, X., additional, Touzot, M., additional, Rabot, N., additional, Thervet, E., additional, Aouba, A., additional, and Karras, A., additional

Published
2021
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48. Évaluation en vie réelle du schéma de corticothérapie à dose réduite selon l’étude PEXIVAS au cours de la granulomatose avec polyangéite et de la polyangéite microscopique

Author

Nagle, S., Nguyen, Y., Puéchal, X., Titeca-Beauport, D., Crépin, T., Mesbah, R., Boudhabhay, I., Pugnet, G., Lebas, C., Néel, A., Karras, A., Hachulla, E., Woessner, J., Borie, R., Vinzio, S., Gouin, J.B., Melboucy-Belkhir, S., Goulenok, T., Guerry, M.J., and Terrier, B.

Published
2023
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49. Plasma Exchange and Glucocorticoids in Severe ANCA-Associated Vasculitis

Author

Walsh, M, Merkel, PA, Peh, C-A, Szpirt, WM, Puéchal, X, Fujimoto, S, Hawley, CM, Khalidi, N, Floßmann, O, Wald, R, Girard, LP, Levin, A, Gregorini, G, Harper, L, Clark, WF, Pagnoux, C, Specks, U, Smyth, L, Tesar, V, Ito-Ihara, T, de Zoysa, JR, Szczeklik, W, Flores-Suárez, LF, Carette, S, Guillevin, L, Pusey, CD, Casian, AL, Brezina, B, Mazzetti, A, McAlear, CA, Broadhurst, E, Reidlinger, D, Mehta, S, Ives, N, Jayne, DRW, Walsh, M, Merkel, PA, Peh, C-A, Szpirt, WM, Puéchal, X, Fujimoto, S, Hawley, CM, Khalidi, N, Floßmann, O, Wald, R, Girard, LP, Levin, A, Gregorini, G, Harper, L, Clark, WF, Pagnoux, C, Specks, U, Smyth, L, Tesar, V, Ito-Ihara, T, de Zoysa, JR, Szczeklik, W, Flores-Suárez, LF, Carette, S, Guillevin, L, Pusey, CD, Casian, AL, Brezina, B, Mazzetti, A, McAlear, CA, Broadhurst, E, Reidlinger, D, Mehta, S, Ives, N, and Jayne, DRW

Abstract

Death from any cause or ESKD occurred in 100 of 352 patients (28.4%) in the plasma-exchange group and in 109 of 352 patients (31.0%) in the control group (hazard ratio, 0.86; 95% confidence interval [CI], 0.65 to 1.13; P=0.27). The results were similar in subgroup analyses and in analyses of secondary outcomes. We also assessed the noninferiority of a reduced-dose regimen of glucocorticoids to a standard-dose regimen, using a noninferiority margin of 11 percentage points. Death from any cause or ESKD occurred in 92 of 330 patients (27.9%) in the reduced-dose group and in 83 of 325 patients (25.5%) in the standard-dose group (absolute risk difference, 2.3 percentage points; 90% CI, −3.4 to 8.0), which met the criterion for noninferiority. Serious infections at 1 year were less common in the reduced-dose group than in the standard-dose group (incidence rate ratio, 0.69; 95% CI, 0.52 to 0.93), but other secondary outcomes were similar in the two groups.

Published
2020

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