Plasma exchange in Goodpasture's syndrome.
The clinical course and levels of anti-glomerular basement membrane (GBM) antibody were compared in 20 patients with Goodpasture's syndrome treated with plasma exchange and immunosuppression (8 patients), immunosuppression alone (4 patients) or no specific therapy (8 patients). There was a more rapid fall in the level of anti-GBM antibody and pulmonary hemorrhage was less protracted in the 8 patients treated with plasma exchange and immunosuppression. In this group, 1 patient who presented with severe renal failure showed a marked improvement of renal function and there was no progression of disease in the 4 with milder renal involvement. 2 of the 4 patients treated with immunosuppression alone, and only 2 of the 8 patients who received no specific therapy, maintained normal renal function. In the group which received no specific therapy, 1 of the 6 patients who progressed to renal failure had mild renal involvement initially. There was a significant correlation between the level of anti-GBM antibody and the severity of the morphological changes seen at renal biopsy but not between the level of anti-GBM antibody and the severity of lung hemorrhage. The course and outcome of the disease in those patients not treated, or treated with immunosuppression alone, was better than that described in early reports of this disease, while those patients with plasma exchange and immunosuppression fared even better. An adequately stratified controlled trial of immunosuppression and plasma exchange versus immunosuppression alone is in order.
D000293:Adolescent; D000328:Adult; D019867:Anti-Glomerular Basement Membrane Disease / Q000276:immunology / Q000628:therapy*; D001323:Autoantibodies / Q000032:analysis; D001485:Basement Membrane / Q000276:immunology; D002102:Cadaver; D005260:Female; D006801:Humans; D007075:Immunoglobulin M / Q000378:metabolism; D007678:Kidney Glomerulus / Q000276:immunology; D016030:Kidney Transplantation; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange
D001323:Autoantibodies; D007075:Immunoglobulin M