Anti-GBN antibody disease is a disorder assosiated with the autoantibody to noncollagenous doain of α3 chain of type IV collagen of glomerularbasement membrane and usually characterizsed by rapidly progressive exracapillary prolifeative
glomerulonephritis with to 80% pulmonary hemorrhage.
A 53-year-old female was admitted to Korea Anam hosptial because of gross hematuria and general edema. On physical examination, the patient who was pale showed 1+ peripheral edema. Serm BUN was 62mg/dl, Crd 10.8mg/dl, Hb 8.8g/dl. Urinalysis
showed
10-15
red blood cells and 20-25 white blood cells/high-power field. The tests for serum IgA, IgG, IgM, C3, C4, ANCA, RF cryoglobulin, FANA, HIV, HBsAg, anti-HCV were either negative or normal. Circulating anti-GBM antibodies which were detected by
enzyme
immunoassay were 2787 units (normal <10 units). The renal biopsy showed the crescents in the glomeruli and immunofluorescence revealed liner staining with IgG along GBM. She was managed with methylprednisolon 1.0g/day of 3days followed by oral
prednisolone 1mg/kg and 8 times of plasmapheresis. Clinical course of the patient was rapidly progressive and her renal function was not restored until now and she has been treated with maintenace hemodialysis. We report a case of anti-GBM
antibody
disease proved with circulating anti-GBM antibody in aserum with review of the literratures in Korea.
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