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Objective: The purpose of this study was to describe the clinical features of AAV and identify possible risk factors for end-stage renal disease (ESRD) and mortality in AAV based on the experience of a single center in China. Methods: A prospective cohort study of AAV was conducted based on data acquired by the Chinese Rheumatism Data Center (CRDC). The cohort involved 140 patients diagnosed with AAV in the Second Affiliated Hospital of Nanchang University from January 2013 to April 2022. Clinical characteristics and prognostic data were prospectively collected. The patients were divided into an ESRD group and a non-ESRD group, a death group and a survival group, Then, univariate and multivariate COX regression models were used to determine the risk factors associated with ESRD and mortality by AAV. Results: Multivariate Cox regression results showed that high initial serum creatinine (hazard ratio (HR) = 1.001, 95% confidence interval (CI): 1.000–1.002, P = 0.024), high initial Birmingham vasculitis activity score (BVAS) (HR = 1.081, 95% CI: 1.027–1.138, P = 0.003), and the need for dialysis treatment (HR = 4.918, 95% CI: 1.727–14.000, P = 0.003) were independent risk factors for the progression of ESRD in AAV patients. Multivariate Cox regression results showed that alveolar hemorrhage (HR = 3.846, 95% CI: 1.235–11.973, P = 0.020), interstitial lung disease (HR = 4.818, 95% CI: 1.788–12.982, P = 0.002), and low initial estimated glomerular filtration rate (EGFR) (HR = 0.981, 95% CI: 0.968–0.995, P = 0.009) were independent risk factors for the prediction of death in AAV patients. Conclusion: These findings suggest that high initial serum creatinine, a high initial BVAS score, and the need for dialysis were independent risk factors for the progression of ESRD in AAV patients. Alveolar hemorrhage, interstitial lung disease, and low initial EGFR were independent risk factors for death. Key Points • The risk factors for ESRD in AAV determined in this study are high initial serum creatinine, a high initial BVAS score, and the need for dialysis. • The risk factors for mortality in AAV are alveolar hemorrhage, interstitial lung disease, and low initial EGFR. [ABSTRACT FROM AUTHOR]