A case of Goodpasture syndrome with diffuse pulmonary hemorrhage / 대한내과학회지

ABSTRACT

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 alpha3 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.