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

Publication

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

Journal Paper/Review - Jan 1, 2021

Papo Matthias, 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 Loic, Emmi Giacomo, Vaglio Augusto, Terrier Benjamin, Jayne David, Barba Thomas, 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, French Vasculitis Study Group and the EGPA European Study Group

Citation

Papo M, 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, Jayne D, Barba T, 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, French Vasculitis Study Group and the EGPA European Study Group. Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Rheumatology (Oxford) 2021; 60:4355-4360.

Type

Journal Paper/Review (English)

Journal

Rheumatology (Oxford) 2021; 60

Publication Date

Jan 1, 2021

Issn Electronic

1462-0332

Pages

4355-4360

Brief description/objective

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.