A Case of Renal Transplantation in a Patient with Goodpasture's Syndrome / 대한이식학회지

ABSTRACT

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.