Titre :

Comparative study of granulomatosis with polyangiitis subsets according to ANCA status: data from the French Vasculitis Study Group Registry.

Auteur(s) :

Puéchal, Xavier [Auteur]
Service de médecine interne et centre de référence des maladies rares [CHU Cochin]
Iudici, Michele [Auteur]
Service de médecine interne et centre de référence des maladies rares [CHU Cochin]
Pagnoux, Christian [Auteur]
Service de médecine interne et centre de référence des maladies rares [CHU Cochin]
Cohen, Pascal [Auteur]
Service de médecine interne et centre de référence des maladies rares [CHU Cochin]
Hamidou, Mohamed [Auteur]
Hôtel-Dieu de Nantes
Aouba, Achille [Auteur]
Université de Caen Normandie [UNICAEN]
Lifermann, François [Auteur]
Centre Hospitalier de Dax
Ruivard, Marc [Auteur]
CHU Clermont-Ferrand
Aumaître, Olivier [Auteur]
CHU Clermont-Ferrand
Bonnotte, Bernard [Auteur]
Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand [CHU Dijon]
Maurier, François [Auteur]
Le Gallou, Thomas [Auteur]
Hachulla, Eric [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Karras, Alexandre [Auteur]
Hôpital Européen Georges Pompidou [APHP] [HEGP]
Khouatra, Chahéra [Auteur]
Université Claude Bernard Lyon 1 [UCBL]
Jourde-Chiche, Noémie [Auteur]
CHU Marseille
Viallard, Jean-François [Auteur]
Centre Hospitalier Universitaire de Bordeaux [CHU Bordeaux]
Blanchard-Delaunay, Claire [Auteur]
Centre Hospitalier Georges Renon [Niort] [CH Georges Renon Niort]
Godmer, Pascal [Auteur]
Université de Bretagne Sud - Vannes [UBS Vannes]
Le Quellec, Alain [Auteur]
Université de Montpellier [UM]
Quéméneur, Thomas [Auteur]
Centre hospitalier [Valenciennes, Nord]
De Moreuil, Claire [Auteur]
Hôpital de la Cavale Blanche - CHRU Brest [CHU - BREST ]
Mouthon, Luc [Auteur]
Service de médecine interne et centre de référence des maladies rares [CHU Cochin]
Terrier, Benjamin [Auteur]
Centre de référence des maladies auto-immunes systémiques rares d'Île-de-France / National Reference Center for Rare Systemic Autoimmune Diseases
Guillevin, Loïc [Auteur]
Service de médecine interne et centre de référence des maladies rares [CHU Cochin]

Titre de la revue :

RMD Open

Nom court de la revue :

RMD Open

Numéro :

8

Date de publication :

2022-03-19

ISSN :

2056-5933

Mot(s)-clé(s) en anglais :

granulomatosis with polyangiitis
systemic vasculitis
autoimmune diseases
autoimmunity

Discipline(s) HAL :

Sciences du Vivant [q-bio]

Résumé en anglais : [en]

Objective To investigate whether antineutrophil cytoplasm antibody (ANCA)-negative and myeloperoxidase (MPO)-ANCA–positive granulomatosis with polyangiitis (GPA) differ from proteinase-3 (PR3)-ANCA–positive GPA. Methods ...
Lire la suite >Objective To investigate whether antineutrophil cytoplasm antibody (ANCA)-negative and myeloperoxidase (MPO)-ANCA–positive granulomatosis with polyangiitis (GPA) differ from proteinase-3 (PR3)-ANCA–positive GPA. Methods Diagnostic characteristics and outcomes of newly diagnosed French Vasculitis Study Group Registry patients with ANCA-negative, MPO-ANCA–positive or PR3-ANCA–positive GPA satisfying American College of Rheumatology criteria and/or Chapel Hill Conference Consensus Nomenclature were compared. Results Among 727 GPA, 62 (8.5%) were ANCA-negative, 119 (16.4%) MPO-ANCA–positive and 546 (75.1%) PR3-ANCA–positive. ANCA-negative patients had significantly (p<0.05) more limited disease (17.7% vs 5.8%) and less kidney involvement (35.5% vs 58.9%) than those PR3-ANCA–positive or MPO-ANCA–positive, with comparable relapse-free (RFS) and overall survival (OS). MPO-ANCA–positive versus PR3-ANCA–positive and ANCA-negative patients were significantly more often female (52.9% vs 42.1%), older (59.8 vs 51.9 years), with more frequent kidney involvement (65.5% vs 55.2%) and less arthralgias (34.5% vs 55.1%), purpura (8.4% vs 17.1%) or eye involvement (18.5% vs 28.4%); RFS was similar but OS was lower before age adjustment. PR3-positive patients’ RFS was significantly lower than for ANCA-negative and MPO-positive groups combined, with OS higher before age adjustment. PR3-ANCA–positivity independently predicted relapse for all GPA forms combined but not when comparing only PR3-ANCA–positive versus MPO-ANCA–positive patients. Conclusions Based on this large cohort, ANCA-negative versus ANCA-positive patients more frequently had limited disease but similar RFS and OS. MPO-ANCA–positive patients had similar RFS but lower OS due to their older age. PR3-ANCA–positive GPA patients’ RFS was lower than those of the two other subsets combined but that difference did not persist when comparing only PR3 versus MPO-ANCA–positive patients.Lire moins >

Langue :

Anglais

Audience :

Internationale

Vulgarisation :

Non

Établissement(s) :

Université de Lille
Inserm
CHU Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Date de dépôt :

2024-01-12T02:02:25Z
2024-03-14T09:03:11Z