HAL will be down for maintenance from Friday, June 10 at 4pm through Monday, June 13 at 9am. More information
Skip to Main content Skip to Navigation
Journal articles

Granulomatosis with polyangiitis: Study of 795 patients from the French Vasculitis Study Group registry

Abstract : Objective: To describe the characteristics and long-term outcomes of patients with granulomatosis with polyangiitis (GPA) from the French Vasculitis Study Group database. Methods: Patients' clinical and laboratory characteristics, Birmingham Vasculitis Activity Score (BVAS)-assessed disease activity, malignancies, opportunistic infections, and vital status were collected at diagnosis and each visit. Estimated probabilities and predictors of overall (OS) and relapse-free survival (RFS) were analyzed by Cox regression. Results: We enrolled 795 newly diagnosed patients, followed for a median of 3.5 years. Initial clinical manifestations involved ear, nose & throat (ENT; 80%), lungs (68%) and kidneys (56%). Among the 728 available ELISA results, 75.0% were PR3-ANCA-positive, 16.5% MPO-ANCA-positive and 62 (8.5%) ANCA-negative. Relapses occurred in 394 (50%) patients, involving ≥1 organ(s) affected at onset in 179 (46%), mainly ENT, lungs and kidneys, with mean BVAS 10.2 points below that at diagnosis (p<0.001). Five- and 10-year RFS rates were 37% and 17%, respectively. PR3-ANCA-positivity independently predicted relapse (p = 0.05) and prolonged survival (p = 0.038). OS-but not RFS-improved significantly over time (p<0.001); 10-year OS reached 88.2% (95% CI 83.9 to 92.7) for the 660 patients diagnosed after 2000. Infections were the main causes of death. Malignancy or opportunistic infection each occurred in ≤5% of the patients. Conclusion: Survival has improved dramatically over the last decades but the high relapse rate remains a major concern for GPA patients, once again stressing the need for therapeutic strategy optimization to lower it. PR3-ANCA-positivity was associated with increased probability of relapse and survival.
Document type :
Journal articles
Complete list of metadata

Contributor : Equipe Hal Université Paris Cité Connect in order to contact the contributor
Submitted on : Friday, June 11, 2021 - 10:28:41 AM
Last modification on : Thursday, April 7, 2022 - 2:28:02 PM




Michele Iudici, Christian Pagnoux, Delphine Courvoisier, Pascal Cohen, Mohamed Hamidou, et al.. Granulomatosis with polyangiitis: Study of 795 patients from the French Vasculitis Study Group registry. Seminars in Arthritis and Rheumatism, WB Saunders, 2021, 51 (2), pp.339-346. ⟨10.1016/j.semarthrit.2021.02.002⟩. ⟨hal-03257789⟩



Record views