Mortality in systemic necrotizing vasculitides: A retrospective analysis of the French Vasculitis Study Group registry - Archive ouverte HAL Access content directly
Journal Articles Autoimmunity Reviews Year : 2018

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

François Maurier
  • Function : Author
Olivier Aumaitre
  • Function : Author
Thomas Quemeneur
  • Function : Author
Jean-François Subra
  • Function : Author
Pascal Godmer
  • Function : Author
Eric Hachulla
Jean-Christophe Lega

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: \textless1980, 1980-1989, 1990-1999, 2000-2010, and >= 2010. The causes of death were classified as vasculitis, infection, cardiovascular, malignancy, miscellaneous, or unknown. 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 \textless 0.001). Periods of diagnosis, age, and male gender were independently associated with a poor survival with a non-significant difference between vasculitis. The incidence of mortality between the 1980s and after 2010 significantly decreased for vasculitis-related (p = 0.03) and cardiovascular-related deaths (p = 0.04). Incidence of death by infection remained stable between the 1980s and the 2000s but no death by infection occurred after 2010. The incidence of death by malignancy remained stable over time. 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.
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hal-02338044 , version 1 (29-10-2019)

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Sabine Jardel, Xavier Puéchal, Alain Le Quellec, Christian Pagnoux, Mohamed Hamidou, et al.. Mortality in systemic necrotizing vasculitides: A retrospective analysis of the French Vasculitis Study Group registry. Autoimmunity Reviews, 2018, 17 (7), pp.653--659. ⟨10.1016/j.autrev.2018.01.022⟩. ⟨hal-02338044⟩
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