Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a common disease with high mortality. Kidney involvement in AAV commonly performances as ANCA-associated glomerulonephritis (AAGN). We aimed to identify the risk factors for mortality and end-stage renal disease(ESRD) within 6 months since diagnosis in AAGN patients. A total of 350 AAGN patients were enrolled in our center between 2004 and 2017 retrospectively. We analyzed the demographic, clinical and follow-up data. Factors for mortality and ESRD were investigated with univariate and multivariate Cox regression models. The median follow-up time was 60.8 (IQR 31.2, 84.5) months and 40 (11.4%) patients died within the first 6 months. In the multivariate analysis, age ≥ 65 years (HR = 2.245, 95%CI 1.085-4.645, P = 0.029), high leukocyte counts (HR = 1.089, 95%CI 1.015-1.168, P = 0.018), high Birmingham Vasculitis Activity Score (BVAS) (HR = 1.089, 95%CI 1.017-1.165, P = 0.014), infection (HR = 2.023, 95%CI 1.013-4.042, P = 0.046) and low serum albumin (HR = 0.916, 95%CI 0.845-0.992, P = 0.030) were independent risk factors for all-cause mortality in the first 6 months. A total of 95 patients reached ESRD within the first 6 months. The renal survival rate was 72.9% at 6 months. Multivariate analysis showed that high BVAS (HR = 1.198, 95%CI 1.043-1.376, P = 0.011), high daily urine protein (HR = 1.316, 95%CI 1.046-1.656, P = 0.019) and low eGFR (HR = 0.877, 95%CI 0.804-0.957, P = 0.003) were independent risk factors for ESRD. The mortality and ESRD rates were high in the first 6 months for AAGN patients. High disease activity evaluated by BVAS impacted both on patients' survival and renal survival, while over 65 years of age and infection were risk factors for mortality.
Keywords: Anti-neutrophil cytoplasmic antibody; End-stage renal disease; Kidney; Mortality; Vasculitis.