Abstract / Summary
Abstract Introduction: ANCA-associated vasculitis (AAV) is a heterogeneous disease associated with substantial morbidity and mortality; however, data integrating both mortality and kidney outcomes within real-world cohorts remain limited. Objective: To evaluate mortality and kidney outcomes in AAV and to identify clinical determinants of first-year and overall mortality as well as progression to end-stage kidney disease (ESKD). Methods: Medical records of patients with AAV from two tertiary referral centers were retrospectively reviewed. Kidney survival and mortality were analyzed using Kaplan–Meier and Cox proportional hazards models. Results: A total of 248 patients (54% male; mean age 51.6 ± 15.6 years) were included. Kidney involvement was present in 73% of patients, and 33% of patients with kidney involvement progressed to ESKD. MPO-ANCA–positive patients had shorter kidney survival ( p = 0.037). The 1- and 5-year overall survival rates were 90.2% and 81.9%, respectively. In multivariable analyses, older age at diagnosis and lower baseline eGFR were consistently associated with higher mortality. Higher serum creatinine was independently associated with ESKD, while hemodialysis requirement at diagnosis was independently associated with ESKD in an alternative model. For first-year mortality, pulmonary and cardiac involvement were also independently associated with mortality, whereas baseline BVAS was independently associated with overall mortality in an alternative model. Conclusion: In this real-world cohort, mortality and kidney outcomes in AAV were primarily associated with baseline kidney function and age. These findings highlight the importance of baseline clinical and renal assessment for mortality and kidney outcome stratification in AAV.