THE FIRST CASE OF GOODPASTURES-DISEASE IN KOREA PROVED WITH POSITIVE CIRCULATING ANTI-GBM ANTIBODIES (AB)

초록

Goodpasture's disease is characterized by renal symptoms, usually a rapidly progressive extracapilary proliferative glomerulonephritis without pulmonary hemorrhage. The pathophysiologic mechanism of this disease is circulating antibody to glomerular basement membrane antigen (anti- GBM Ab) binding to kidney and lung. With the development of a molecular biologic technique, the primary target of anti-GBM antibody is identified as a 28-kD noncollagenous C-terminus of the a3 chain of type IV collagen known as NCI. Recently, we experienced the first case of Goodpasture's disease in Korea, which manifested as rapidly progressive glomerulonephritis. This patient is a 53 year old female who developed a generalized edema and anuria. On physical examination, she appeared to be pale with peripheral pitting edema. Laboratory values were as follows: hct 24.9%, BUN 62 mg/dl, and Cr 11.8 mg/dl. Serologic markers such as HBs Ag, CRP, RF, FANA, ANCA and ciyoglobulin were all negative. Urine analysis showed 3+ for protein and RBC 10—15/HPF. The patient's course progressively deteriorated, steroid pulse therapy failed to avert the fulminating condition, and plasmapheresis was done for 3 weeks. Renal biopsy revealed a diffuse proliferative, crescent glomerulonephritis (100%) with severe tubulointerstitial edema and inflammatory cell infiltration. Direct immunofluorescent staining revealed a linear IgG and C3 deposits along glomerular basement membrane. Circulating anti-GBM Ab using radioimmunoassay was strongly positive (titer 1:2787 unit). Renal dysfunction was not restored, and she is currently being treated withmaintenance hemodialysis.