Over the last 16 years we have treated 40 patients with a renal pathological diagnosis of anti-glomerular basement membrane (anti-GBM) disease. The age of presentation ranged from 18 years to 76. Males and females were equally affected. The most common presenting symptoms were anorexia and uremia. Fifty percent of the patients were oliguric at presentation and required dialysis within 24 h, while the mean serum creatinine (Cr) at presentation was 5.1±6.8 mg/dL. Sixty-two percent of the patients demonstrated evidence of pulmonary involvement. Hematuria was universally present and was macroscopic in 35% of cases. Eight-two percent of the biopsies demonstrated greater than 80% crescents. Eight patients retained independent renal function after immunosuppression with cyclophosphamide, prednisone, and plasmapheresis. No patient who presented with a Cr greater than 5 mg/dL retained renal function. Using a logistic regression model the only clinical, biochemical, or pathological factors at presentation that significantly predicted eventual need for dialysis were the serum creatinine and percentage of glomeruli with crescents. Ten patients underwent cadaveric renal transplantation, and 7 have a functioning graft for a mean of 8.2 years. None have shown evidence of recurrence of anti-GBM disease in the graft. We conclude that anti-GBM disease is an important cause of renal failure and that its prognosis is directly related to the degree of renal failure at presentation. Efforts need to be made to diagnose this condition earlier in its natural history if its prognosis is to be improved.