Goodpasture 증후군은 우리나라에서는 극히 드문 질환으로 급성신부전과 폐출혈이 있으면서 항 사구체 기저막 항체가 증명될 경우 확진되며 혈액투석, 혈장교환술, 면역흡착법 등의 방법을 통해 항 사구체 기저막 항체의 역가를 감소시키고 면역억제제를 투여하여 항체 형성을 억제할 경우 임상 증상의 빠른 호전을 기대해 볼 수 있다. 저자들은 이전에 보고된 경우가 대량 폐출혈을 동반했던 것과 다르게 미만성 폐출혈 양상이었으며 신질환에 있어서는 좋지 않은 예후를 보였던 Goodpasture 증후군 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

Goodpasture syndrome is an autoimmune disease with a triad of acute renal failure due to rapidly progressive glomerulonephritis (RPGN), pulmonay hemorrhage and circulating anti-glomerular basement membrane antibody (anti-GBM Ab). It was commonly reported from Europe in male with a peak incidence in their 20's. If patients are affected with the disease, relief of symptoms can be expected by eliminating the anti-GBM Ab from the circulatory system through hemodialysis, plasmapheresis and immunoadsorption. However, if the diagnosis or treatment is delayed, the patients usually die from massive pulmonary hemorrhage. It has been revealed that the main target of anti-GBM Ab's is NC1 domain on the α3 chain of type IV collagen. Currently there are many studies underway using this information as a basis to identify the pathogenesis of Goodpasture syndrome and to develop new therapeutic approach. The patient was a 20-year-old male with a chief complaint of edema. Unlike patients in the two previous cases, reported in Korea who had massive hemorrhage, he showed diffuse pulmonary hemorrhage which improved in only one week by hemodialysis. Renal biopsy demonstrated crescents in over 90% of glomeruli and showed signs of acute renal failure due to RPGN, with 618 U/mL (normal range <19.9 U/mL) of anti-GBM Ab titer.(Korean J Med 63:85-91, 2002)

Goodpasture syndrome is an autoimmune disease with a triad of acute renal failure due to rapidly progressive glomerulonephritis (RPGN), pulmonay hemorrhage and circulating anti-glomerular basement membrane antibody (anti-GBM Ab). It was commonly reported from Europe in male with a peak incidence in their 20's. If patients are affected with the disease, relief of symptoms can be expected by eliminating the anti-GBM Ab from the circulatory system through hemodialysis, plasmapheresis and immunoadsorption. However, if the diagnosis or treatment is delayed, the patients usually die from massive pulmonary hemorrhage. It has been revealed that the main target of anti-GBM Ab's is NC1 domain on the α3 chain of type IV collagen. Currently there are many studies underway using this information as a basis to identify the pathogenesis of Goodpasture syndrome and to develop new therapeutic approach. The patient was a 20-year-old male with a chief complaint of edema. Unlike patients in the two previous cases, reported in Korea who had massive hemorrhage, he showed diffuse pulmonary hemorrhage which improved in only one week by hemodialysis. Renal biopsy demonstrated crescents in over 90% of glomeruli and showed signs of acute renal failure due to RPGN, with 618 U/mL (normal range <19.9 U/mL) of anti-GBM Ab titer.(Korean J Med 63:85-91, 2002)

Goodpasture syndrome, Anti-glomerular basement membrane antibody, Acute renal failure, Pulmonary hemorrhage