Damage accrual and predictors of mortality in ANCA‑associated vasculitis: a retrospective observational study

In this study, we aimed to evaluate the factors affecting the development of damage and mortality in patients with AAV
treated at our tertiary referral center. This retrospective study included data on patients with AAV who fulfilled the Chapel
Hill Consensus Conference (CHCC) criteria. Patients were divided into c-ANCA/PR3( +) and p-ANCA/MPO ( +) groups
based on ANCA immunofluorescence and/or ELISA results, and relapse, damage, and mortality data were compared across
the groups. Data from 254 patients (n = 136, 53.5% female) were included in the analysis. Clinical diagnosis was GPA in
186 (73.2%) and MPA in 68 (26.8%) patients. During the follow-up, 217 of 242 (89.7%) patients developed damage, and the
median VDI score of the cohort was 2 (IQR: 2). VDI scores were higher in the first period (1997–2011) than in the second
period (2011–2021) in the entire cohort (p = 0.012) and in patients with GPA compared with MPA (p = 0.034). Five-year and
overall survival rates were 88.1% and 80.3% in the entire cohort; 87.8% and 81% in c-ANCA/PR3 ( +); 86.2% and 76% in
p-ANCA/MPO ( +) (Log-Rank: p = 0.35); 91% and 84.5% in GPA; 81% and 67.2% in MPA patients (Log-Rank: p < 0.001).
Development of malignancy, severe infection, and active/persistent disease after the induction phase were associated with
higher mortality in patients with AAV. In our AAV cohort, permanent organ damage was detected in the majority of the
patients. Although the median VDI score decreased over time, mortality did not change.

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