Goodpasture症候群に施行した献腎移植の1例

Abstract

19歳男.血痰を自覚し, 急速進行性糸球体腎炎の所見を呈した.腎生検によりGoodpasture症候群と診断した.ステロイドパルス療法と血漿交換により症状の改善が認められた.しかし, 腎機能は回復せず透析導入となった.男をドナーとして献腎移植術を施行した.免疫抑制剤はシクロスポリン, メチルプレドニゾロン, アザチオプリン, ミゾリビンで開始し, 抗リンパ球グロブリンを14日間使用した.術後6日目には透析離脱となった.移植後1年目の定期的腎生検では拒絶反応, 原病再発の所見は認められなかった

A 19-year-old man with a history of histologically-proven Goodpasture's syndrome (hemoptysis, rapidly progressive glomerulonephritis, and positive anti-glomerular basement membrane (anti-GBM) antibody) was maintained on hemodialysis for 21 months. After steroid pulse therapy and plasmapheresis, his anti-GBM antibody disappeared. His stable condition on dialysis and a session of plasmapheresis prior to surgery allowed him to undergo cadaveric renal transplantation from a 34-year-old man. The blood type was identical (group A and Rh (+)), and there was 1 and 0 mismatch of HLA class 1 and 2, respectively. The initial immunosuppressants included cyclosporine, methylprednisolone, mizoribine, azathioprine, and anti-lymphocyte globulin (ALG). The transplanted kidney regained function on day 6 and he was free from hemodialysis. Circulating anti-GBM antibody was negative after surgery. The graft has functioned well for almost 4 years after transplantation without any episodes of renal or pulmonary complications. To the best of our knowledge, this is the first report of renal transplantation for Goodpasture's syndrome in the Japanese literature.