Mortality in systemic necrotizing vasculitides: A retrospective analysis of the French Vasculitis Study Group registry

Abstract Objective The aim of the study was to describe the evolution of mortality and cause-specific mortality over time in patients with systemic necrotizing vasculitides (SNV), including polyarteritis nodosa (PAN), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Methods Patients with SNV from the French Vasculitis Study Group registry were divided into 5 groups according to the date of diagnosis: Results Among the 2217 patients included (PAN 16.1%, GPA 41.7%, EGPA 22.6%, MPA 19.6%), overall incidence of death was 2.26 per 100 person-years. The overall survival improved during each period considered. The 5-year survival rate increased from 72.2% (95% confidence interval [CI] 59.7–87.2) for patients diagnosed before 1980 to 94.5% (95% CI 90.4–98.8) after 2010 (p  Conclusion Overall survival of SNV patients has improved since the 1980s with the decrease of vasculitis- and cardiovascular-related deaths, but cancer-related mortality remained stable. These results highlight malignancy as the current target to improve the overall prognosis.