Background: It has been reported that after patients with antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) progress to end-stage renal disease (ESRD), they are less likely to experience relapse of vasculitis. However, we encountered a few patients with ESRD suffering severe pulmonary hemorrhage caused by relapse of AAV. The current study presents our observation on these patients.
Methods: Of 198 consecutive patients with AAV with follow-up data in our center, 66 progressed to ESRD during follow-up. Clinical and laboratory data were collected and retrospectively analyzed.
Results: Among the 66 patients with ESRD, 5 experienced severe pulmonary hemorrhage. They had positive serum perinuclear ANCA and myeloperoxidase ANCA and were diagnosed as microscopic polyangiitis. All 5 patients achieved remission after initial induction therapy. The average duration of follow-up was 47.0 (range 8.0-98.0) months. After progressing to ESRD and starting hemodialysis, these patients experienced severe pulmonary hemorrhage within 9.0 (range 2.0-23.0) months. After immunosuppressive therapy, pulmonary hemorrhage ceased in 4 patients, and the other died of respiratory failure.
Conclusions: Severe pulmonary hemorrhage can occur in ESRD patients with AAV. Disease activity and relapses of AAV should be monitored even after patients progress to ESRD.