肺出血を伴ったp-ANCA陽性の急速進行性糸球体腎炎の1例

Abstract

We report a case of rapidly progressive glomerulonephritis (RPGN) with perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) who presented with alveolar hemorrhage during the clinical course. A 72-year-old woman was admitted to our hospital for close examination of acute renal failure, proteinuria and hematuria. Laboratory data on admission showed massive proteinuria (2.1g/day), elevated serum creatinine (3.4mg/ dl), and p-ANCA positive. Anti-glomerular basement membrane antibody was negative. Renal biopsy revealed necrotizing crescentic glomerulonephritis without deposition of immunoglobulins. She required hemodialysis soon after admission. Combined therapy including prednisolone, cyclophosphamide, heparin and dipyridamole was performed, but was ineffective. Furthermore, alveolar hemorrhage appeared 36 days after admission. She died of massive pulmonary bleeding. One therefore should pay particular attention to alveolar hemorrhage in patients with p-ANCA positive RPGN.