Severe pulmonary hemorrhage in patients with end-stage renal disease in antineutrophil cytoplasmic autoantibody-associated vasculitis.

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.