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

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TitreSignificance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
Type de publicationJournal Article
Year of Publication2021
AuteursPapo M, Sinico RA, Teixeira V, Venhoff N, Urban M-L, 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-E, Bonnotte B, Durel C-A, Puechal X, Mouthon L, Guillevin L, Emmi G, Vaglio A, Terrier B, Grp FVasculitis, Grp EGPAEuropean S
JournalRHEUMATOLOGY
Volume60
Pagination4355-4360
Date PublishedSEP
Type of ArticleArticle
ISSN1462-0324
Mots-clésANCA, Churg-Strauss, vasculitis
Résumé

{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%

DOI10.1093/rheumatology/keaa805