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KMID : 0361120020160020258
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Korean Journal of Transplantation
2002 Volume.16 No. 2 p.258 ~ p.261
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A Case of Renal Transplantation in a Patient with Goodpasture's Syndrome
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박민/Min Park
전지민/김성민/최윤석/이종명/신용훈/박용기/김중경/정광진*/Ji Min Jeon/Seong Min Kim/Yoon Seog Choi/Jong Myung Lee/Yong Hun Sin/Yong Ki Park/Joong Kyung Kim/Kwang Jin Jeong*
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Abstract
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Goodpasture's syndrome is a clinical complex of anti-GBM (glomerular basement membrane) nephritis and lung hemorrhage. Anti-GBM nephritis is an autoimmune disease in which autoantibodies directed against type Ⅳ collagen induce RPGN (rapid
progressive
glomerulonephropathy) and crescentic glomerulonephritis. 50 to 70% of patients have pulmonary hemorrhage. We have one case of a successful renal transplantation in a patient with Goodpasture's syndrome. A 51 year old male had arrived in the
emergency
room due to dyspnea. 20 days prior to admission he had suffered from fever and then progressively developed nausea and weakness. He underwent hemodialysis with the dual lumen catheter which was inserted in the right internal jugular vein.
Goodpasture's
syndrome was confirmed by the measurement of serum anti-GBM Ab titer, renal biopsy and clinical manifestations of pulmonary hemorrhage. Renal biopsy findings showed diffuse proliferative glomerulonephritis on light microscopy and linear
ribbon-like
deposition of IgG along the GBM. The patient was placed on CAPD on the 30th hospital day. After six months of CAPD, the patient received a renal transplant from a HLA-haploidendical brother. Which was done after negative seroconversion
of
circulating antibody of GBM. With a induction of IL-2 receptor blocker (Basiliximab), cyclosporine-A and prednisone were administered for their immunosuppressants. He had good health for 37 months with excellent graft function.
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KEYWORD
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