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Journal Articles Annals of the Rheumatic Diseases Year : 2018

Long-term efficacy of remission-maintenance regimens for ANCA-associated vasculitides

Benjamin Terrier
Adexandre Karras
  • Function : Author
Chahera Khouatra
  • Function : Author
Pascal Cohen
  • Function : Author
François Maurier
  • Function : Author
Pierre Gobert
  • Function : Author
Claire Blanchard-Delaunay
  • Function : Author
Bernard Bonnotte
Pierre-Louis Carron
  • Function : Author
Marize Ducret
  • Function : Author
Pascal Godmer
  • Function : Author
Olivier Lidove
Nicolas Limal
  • Function : Author
Xavier Puéchal
Luc Mouthon
  • Function : Author


Objective - To compare long-term efficacy of remission-maintenance regimens in patients with newly diagnosed or relapsing antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides. Methods - The 28-month Maintenance of Remission using Rituximab in Systemic ANCA-associated Vasculitis trial compared rituximab with azathioprine to maintain remission in patients with newly diagnosed or relapsing granulomatosis with polyangiitis, microscopic polyangiitis or renal-limited ANCA-associated vasculitis. Thereafter, prospective patient follow-up lasted until month 60. The primary endpoint was the major-relapse rate at month 60. Relapse and serious adverse event-free survival were also assessed. Results - Among the 115 enrolled patients, only one was lost to follow-up at month 60. For the azathioprine and rituximab groups, respectively, at month 60, the major relapse-free survival rates were 49.4% (95% CI 38.0% to 64.3%) and 71.9% (95% CI 61.2% to 84.6%) (p=0.003); minor and major relapse-free survival rates were 37.2% (95% CI 26.5% to 52.2%) and 57.9% (95% CI 46.4% to 72.2%) (p=0.012); overall survival rates were 93.0% (95% CI 86.7% to 99.9%) and 100% (p=0.045) and cumulative glucocorticoid use was comparable. Quality-adjusted time without symptoms and toxicity analysis showed that rituximab-treated patients had 12.6 months more without relapse or toxicity than those given azathioprine (p<0.001). Antiproteinase-3-ANCA positivity and azathioprine arm were independently associated with higher risk of relapse. HRs of positive ANCA to predict relapse increased over time. Conclusion - The rate of sustained remission for ANCA-associated vasculitis patients, following rituximab-based or azathioprine-based maintenance regimens, remained superior over 60 months with rituximab, with better overall survival. Trial registration number - NCT00748644.

Dates and versions

hal-01812523 , version 1 (11-06-2018)



Benjamin Terrier, Christian Pagnoux, Elodie Perrodeau, Adexandre Karras, Chahera Khouatra, et al.. Long-term efficacy of remission-maintenance regimens for ANCA-associated vasculitides. Annals of the Rheumatic Diseases, 2018, 77 (8), pp.1150-1156. ⟨10.1136/annrheumdis-2017-212768⟩. ⟨hal-01812523⟩
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