Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression.

Background: Anti-glomerular basement membrane (GBM) antibody disease is an autoantibody-mediated disorder that usually presents as rapidly progressive glomerulonephritis, often with pulmonary hemorrhage (the Goodpasture syndrome). It is reported that patients with severe renal failure do not generally recover renal function.

Objective: To examine the long-term outcome of severe anti-GBM antibody disease.

Design: Retrospective review of patients treated for confirmed anti-GBM antibody disease over 25 years.

Setting: A tertiary referral center in the United Kingdom.

Patients: 71 treated patients with anti-GBM antibody disease.

Intervention: All patients received plasma exchange, prednisolone, and cyclophosphamide,

Measurements: Patient and renal survival, renal histology, and antibody levels.

Results: Patients who presented with a creatinine concentration less than 500 mu mol/L (5.7 mg/dL) (n = 19) had 100% patient survival and 95% renal survival at 1 year and 84% patient survival and 74% renal survival at last follow-up. In patients who presented with a creatinine concentration of 500 mu mol/L or more (greater than or equal to5.7 mg/dL) (n = 13) but did not require immediate dialysis, patient and renal survival were 83% and 82% at 1 year and 62% and 69% at last follow-up. In patients who presented with dialysis-dependent renal failure (n = 39), patient and renal survival were 65% and 8% at 1 year and 36% and 5% at last follow-up. All patients who required immediate dialysis and had 100% crescents on renal biopsy remained dialysis dependent.

Conclusions: Patients with the Goodpasture syndrome and severe renal failure should be considered for urgent immunosuppression therapy, including plasma exchange, to maximize the chance of renal recovery. Patients needing immediate dialysis are less likely to recover.