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Iudici, Michele; Pagnoux, Christian; Courvoisier, Delphine S.; Cohen, Pascal; Hamidou, Mohamed; Aouba, Achille; Lifermann, François; Ruivard, Marc; Aumaître, Olivier; Bonnotte, Bernard; Maurier, François; Decaux, Olivier; Hachulla, Eric; Karras, Alexandre; Khouatra, Chahéra; Jourde-Chiche, Noémie; Viallard, Jean-François; Blanchard-Delaunay, Claire; Godmer, Pascal; Quellec, Alain Le; Quéméneur, Thomas; de Moreuil, Claire; Régent, Alexis; Terrier, Benjamin; Mouthon, Luc; Guillevin, Loïc; Puéchal, Xavier
Seminars in arthritis and rheumatism, April 2021, 2021-04-00, 20210401, 2021-04, Volume: 51, Issue: 2Journal Article
To describe the characteristics and long-term outcomes of patients with granulomatosis with polyangiitis (GPA) from the French Vasculitis Study Group database. 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. 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. 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.
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