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.
Papo, M., Sinico, R., Teixeira, V., Venhoff, N., Urban, M., Iudici, M., et al. (2021). Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss). RHEUMATOLOGY, 60(9 (September 2021)), 4355-4360 [10.1093/rheumatology/keaa805].
|Citazione:||Papo, M., Sinico, R., Teixeira, V., Venhoff, N., Urban, M., Iudici, M., et al. (2021). Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss). RHEUMATOLOGY, 60(9 (September 2021)), 4355-4360 [10.1093/rheumatology/keaa805].|
|Tipo:||Articolo in rivista - Articolo scientifico|
|Carattere della pubblicazione:||Scientifica|
|Presenza di un coautore afferente ad Istituzioni straniere:||Si|
|Titolo:||Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss)|
|Autori:||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|
SINICO, RENATO ALBERTO (Secondo) [Membro del Collaboration Group]
|Data di pubblicazione:||2021|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1093/rheumatology/keaa805|
|Appare nelle tipologie:||01 - Articolo su rivista|