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

• Published in: Rheumatology (Oxford, England) Vol. 60; no. 9; pp. 4355 - 4360
• Main Authors: 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
• Format: Journal Article
• Language: English
• Published: England Oxford University Press 01.09.2021
• Subjects: Churg-Strauss; ANCA; vasculitis
• ISSN: 1462-0324; 1462-0332
• DOI: 10.1093/rheumatology/keaa805

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.