Abstract
OBJECTIVE: To examine temporal trends in mortality rates in a large nationwide cohort of patients with end-stage renal disease (ESRD) due to granulomatosis with polyangiitis (Wegener's) (GPA-ESRD). METHODS: We identified cases of GPA-ESRD reported in the US Renal Data System between 1995 and 2014, using coding by nephrologists for the cause of ESRD. The cohort was divided into four 5-year subcohorts based on the year of onset of ESRD (1995-1999, 2000-2004, 2005-2009, and 2010-2014) to assess trends in mortality rates and hazard ratios (HRs) for overall death and cause-specific death, adjusting for potential confounders. RESULTS: Between 1995 and 2014, there were 5,929 incident cases of GPA-ESRD. The mortality rate (incidence per 100 patient-years) declined from 19.0 in 1995-1999 to 15.3 in 2010-2014 (P for trend = 0.01). The multivariable-adjusted HR for mortality in the 2010-2014 cohort was 0.77 (95% confidence interval [95% CI] 0.66-0.90) (P for trend < 0.001 versus the 1995-1999 cohort). The corresponding cause-specific HRs for mortality, after accounting for competing risk, were 0.61 (95% CI 0.47-0.80) for CVD-related death and 0.42 (95% CI 0.28-0.63) for death due to infection (P for trend < 0.001). CONCLUSION: In this study of nearly all patients in whom GPA-ESRD developed in the US over 2 decades, we observed significant improvements in mortality among GPA-ESRD patients. The number of cause-specific deaths due to CVD and the number due to infections declined significantly during the study period. These findings are encouraging and likely reflect improved management of both GPA and ESRD.