Granulomatosis with polyangiitis: Study of 795 patients from the French Vasculitis Study Group registry - Université Paris Cité Access content directly
Journal Articles Seminars in Arthritis and Rheumatism Year : 2021

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

Christian Pagnoux
  • Function : Author
Delphine Courvoisier
Pascal Cohen
  • Function : Author
Mohamed Hamidou
  • Function : Author
Achille Aouba
  • Function : Author
François Lifermann
  • Function : Author
Marc Ruivard
  • Function : Author
Olivier Aumaître
  • Function : Author
Bernard Bonnotte
François Maurier
  • Function : Author
Olivier Decaux
  • Function : Author
Eric Hachulla
  • Function : Author
Chahéra Khouatra
  • Function : Author
Noémie Jourde-Chiche
Jean-François Viallard
  • Function : Author
Claire Blanchard-Delaunay
  • Function : Author
Pascal Godmer
  • Function : Author
Alain Le Quellec
  • Function : Author
Thomas Quéméneur
  • Function : Author
Claire de Moreuil
  • Function : Author
Benjamin Terrier
Luc Mouthon
  • Function : Author


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.
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hal-03257789 , version 1 (09-03-2023)


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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, 2021, 51 (2), pp.339-346. ⟨10.1016/j.semarthrit.2021.02.002⟩. ⟨hal-03257789⟩
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