Korean J Med.  2002 Jul;63(1):85-91.

A case of Goodpasture syndrome with diffuse pulmonary hemorrhage

• Kim HS
• Kim HJ
• Hwang YC
• Lee TW
• Kim MJ
• Yang MH
• Sung DW
• Lee SH
• Ihm CG

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.

Keyword

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

MeSH Terms

Acute Kidney Injury
Anti-Glomerular Basement Membrane Disease*
Autoimmune Diseases
Basement Membrane
Biopsy
Collagen Type IV
Diagnosis
Edema
Europe
Glomerulonephritis
Hemorrhage*
Humans
Incidence
Korea
Male
Plasmapheresis
Renal Dialysis
Young Adult
Collagen Type IV