Abstract: SUMMARY Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is usually a multisystem disorder, and pulmonary renal syndrome is a common presentation. Patients with AAV are less likely to experience relapse when they progress to end-stage renal disease (ESRD). We report a rare case of diffuse alveolar hemorrhage (DAH) in relapsing AAV after eight years of haemodialysis. A 58-year-old woman was admitted to our hospital with the chief complaints of dyspnea and hemoptysis accompanied by anemia, fever, fatigue, and weight loss. She had elevated anti-myeloperoxidase (MPO) titer. The computer tomoghraphy showed diffuse alveolar hemorrhage. After the recurrent episode of AAV was diagnosed, she underwent the following therapy: Plasmapheresis was initiated within 24 h after admission, 3 000 mL of plasma was removed per session, and the anticoagulation of citrate was applied during plasmapheresis. Five plasmapheresis treatments were performed, and after three apheresis sessions, the pulmonary hemorrhage ceased. Other treatments included a methylprednisolone bolus, tapered to oral prednisone and cyclophosphamide. Regular hemodialysis was scheduled. These treatments resulted in resolution of the inflammatory symptoms, DAH improved. Her anti-MPO level decreased. The patient was discharged in good condition. AAV with DAH is usually acute at the onset and is generally a condition with high morbidity and substantial mortality. Therefore, prompt diagnosis and aggressive treatment are needed to improve survival.

Key words: Antibodies, antineutrophil cytoplasmic, Vasculitis, Hemodialysis, Hemorrhage, Lung diseases