A Case of Goodpasture's Syndrome with Normal Renal Function / 결핵및호흡기질환

ABSTRACT

Goodpasture's syndrome is a disease that is characterized by hemoptysis, anemia, and glomerulonephritis with renal failure. Goodpasture reported a case of a young man who expired as a result of a pulmonary hemorrhage and glomerulonephritis at the recovery phase after an influenza infection in 1919. In 1958, Stanton et al. described a combined case of these two diseases as Goodpasture's syndrome. Since then, antiglomerular basement membrane antibody(anti-GBM Ab) has been confirmed to play an important role in the mechanism of this syndrome, and it was reported that this syndrome was an autoimmune disease. The triad of alveolar hemorrhage, glomerulonephritis and circulating anti-GBM Ab forms the basis of a diagnosis of Goodpasture's syndrome. When patients are affected by disease, the relief of symptoms can be accomplished by eliminating the anti-GBM Ab from the circulatory system through hemodialysis, plasmapheresis and immunoabsorption. However, the patients usually die from a massive pulmonary hemorrhage when the diagnosis or treatment is delayed. The incidence of Goodpasture's syndrome is common in the western world, but it is extremely rare in Korea with only five cases being reported. In three of these cases, pulmonary hemorrhage and renal failure was the initial manifestation. Therefore, hemodialysis or plasmapheresis were absolutely essential treatments. We report a case of Goodpasture's syndrome in Korea with a normal renal function.