Background The mortality of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is generally greater than for other types of vasculitis. The long-term survival of patients (pts) with ANCA-associated vasculitis in Ukraine is uncertain.
Objectives To determine predictors of survival of pts with ANCA-associated vasculitis.
Methods Outcome data was collected for 49 pts who had been diagnosed with ANCA-associated vasculitis (27 pts with granulomatosis with polyangiitis (GPA), 10 pts with microscopic polyangiitis (MPA) and 12 pts with eosinophilic granulomatosis with polyangiitis) according to the ACR 1990 criteria or the CHCC diagnostic criteria in the two city hospitals in Kyiv between 1972 and 2013. There were 15 males and 34 women aged from 20 to 77 years (median age 48.5±13.8). The duration of disease at time of diagnosis was from 1 to 156 months (mean 22.5±34.3 months). All pts received glucocorticoids, 37 - cytotoxic drugs and 3 – rituximab according to local treatment protocols. Survival was evaluated as a function of the main demographics, clinical and laboratory parameters and Birmingham Vasculitis Activity Score (BVAS) assessed at diagnosis. Statistical analyses used the Kaplan-Meier method and the multivariate Cox proportional hazards regression model.
Results The median duration of follow-up was 62 months and 6 deaths (12.2%) were recorded (4 pts with MPA and 2 pts with GPA). The predominant causes of deaths were renal failure and disease activity. Cumulative survival rates at 1, 3, 5 and 10 years was 91.8, 87.6, 87.6 and 76.7%, respectively. Comparison survival of pts with ANCA-associated vasculitis according to age at onset of the disease and sex revealed that gender and age cumulative survival function didn't differ significantly. According to univariate analysis, a poor prognostic value was attributed to serum creatinine more than 176 umol/l (p=0.0001) and hemorrhagic alveolitis (p=0.01). Other factors (including ear, nose and throat, joints, skin, heart and nervous system involvement, increased ESR, leukocytosis, anemia, ANCA positivity, etc) didn't have influence on the survival curves. Survival curves of groups of pts with different disease activity determined by BVAS were not significantly different (p=0.88). Multivariate analysis showed that presence of increased creatinine levels more than 176 umol/l (HR=3.04, p=0.0001) and hemorrhagic alveolitis (HR=1.94, p=0.02) were independent predictors of poor outcome in pts with GPA and MPA.
Conclusions Cumulative survival of pts with ANCA-associated vasculitis at 1, 3, 5 and 10 years of observation is 92, 88, 88 and 77%, respectively. The increased creatinine levels more than 176 umol/l and presence of hemorrhagic alveolitis are independent predictors of poor survival of pts with GPA and MPA. Vasculitis activity detected by BVAS and other clinical and laboratory parameters no effect on mortality.
Disclosure of Interest None declared