BACKGROUND: Approximately 20% of patients with antineutrophil cytoplasm antibody-associated systemic vasculitis (AASV) develop end-stage renal failure (ESRF). It is not clear whether continuation of immunosuppression, with its associated risks, is beneficial because relapse rates after the development of ESRF are reported to be low. STUDY DESIGN: Case series. SETTING & PARTICIPANTS: Single tertiary-care referral center. 46 patients with AASV who developed ESRF from 1971 to 2004. OUTCOMES & MEASUREMENTS: Treatment, relapse rates before and after dialysis therapy, patient outcome after dialysis therapy, and infection (defined as admission to hospital or intravenous antibiotics) were recorded. RESULTS: Patients with AASV on dialysis therapy had 1- and 5-year survival rates of 82% and 55%, equivalent to current 1- and 5-year survival rates of dialysis patients reported by the UK renal registry, respectively. Infection rates in patients with ESRF were high in those with AASV on dialysis therapy; 106 events in 35 patients (dialysis patients with AASV, 0.89 infections/patient-year; confidence interval [CI], 0.74 to 1.08). Eight of 9 patients who died of infection were receiving immunosuppressive therapy. No patient died of active disease. Relapse rates after dialysis commencement were less than those predialysis (6 relapses in 4 patients; 0.05 relapses/patient-year postdialysis; CI, 0.02 to 0.1 compared with 18 relapses in 11 patients; 0.13 relapses/patient-year predialysis; CI, 0.07 to 0.19). LIMITATIONS: This is a retrospective study spread over 3 decades with no control group. CONCLUSIONS: Patients with AASV and ESRF are less likely to experience relapse than before dialysis therapy. Patients with AASV on dialysis therapy have a high rate of infection. These results may not be applicable to patients with pulmonary involvement.
- microscopic polyangiitis
- antineutrophil cytoplasm antibody (ANCA)-associated vasculitis
- Wegener granulomatosis