Abstract
We report two patients with ANCA associated vasculitis. They presented with renal insufficiency and respiratory distress because of pulmonary hemorrhage, and were seropositive for MPO-ANCA. The histological findings showed pauci-immune crescentic glomerulonephritis. So they were diagnosed as ANCA associated vasculitis.
Case 1 was a 7-year-old female. She was treated with methylprednisolone pulse therapy and followed by oral cyclophosphamide, prednisolone, azathioprine and dipyridamole. However she developed end-stage renal failure after four years, so she has been receiving peritoneal dialysis since then.
Case 2 was a 6-year-old female. She was treated with peritoneal dialysis for acute renal failure and methylprednisolone pulse therapy followed by oral prednisolone for respiratory distress. The pulmonary hemorrhage and the respiratory insufficiency improved. But after several days, pulmonary hemorrhage relapsed, again. At that time, methylprednisolone pulse therapy was non effective and respiratory distress was more severe. So we decided to perform plasma exchange therapy and it was so effective that pulmonary hemorrhage had completely improved.
In conclusion, prognosis of renal survival in ANCA associated vasculitis is still bad but mortality is not so high in pediatric patients. When primary disease is not improved by steroid and immunosuppressive therapy, plasma exchange may be effective for the disease.
