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5 June 2001 | Volume 134 Issue 11 | Pages 1033-1042
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 µmol/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 µmol/L or more (
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.
Author and Article Information
From Imperial College School of Medicine, Hammersmith Hospital, London; University of Edinburgh, Royal Infirmary, Edinburgh; and University of Aberdeen, Institute of Medical Sciences, Aberdeen, United Kingdom.
Acknowledgments: The authors thank all the clinicians who have referred patients and all colleagues who have been involved in patient care or diagnosis at the Hammersmith Hospital over the past three decades, especially Professor Sir Keith Peters and Dr. Martin Lockwood.
Requests for Single Reprints: Jeremy B. Levy, MA, PhD, MRCP, Renal Section, Imperial College School of Medicine, Hammersmith Hospital, Ducane Road, London W12 0NN, United Kingdom.
Current Author Addresses: Drs. Levy and Pusey: Renal Section, Imperial College School of Medicine, Hammersmith Hospital, Ducane Road, London W12 ONN, United Kingdom.
Dr. Turner: Department of Medicine, University of Edinburgh, Royal Infirmary, Lauriston Place, Edinburgh EH3 9YW, United Kingdom.
Dr. Rees: Department of Medicine and Therapeutics, University of Aberdeen, Institute of Medical Sciences, Foresterhill, Aberdeen AB25 2ZD, United Kingdom.
Author Contributions: Conception and design: J.B. Levy, C.D. Pusey.
Analysis and interpretation of the data: J.B. Levy, A.N. Turner, C.D. Pusey.
Drafting of the article: J.B. Levy.
Critical revision of the article for important intellectual content: J.B. Levy, A.N. Turner, A.J. Rees, C.D. Pusey.
Final approval of the article: J.B. Levy, A.N. Turner, A.J. Rees, C.D. Pusey.
Provision of study materials or patients: A.N. Turner, A.J. Rees, C.D. Pusey.
Statistical expertise: J.B. Levy.
Collection and assembly of data: J.B. Levy, C.D. Pusey. ARTICLE
Long-Term Outcome of Anti–Glomerular Basement Membrane Antibody Disease Treated with Plasma Exchange and Immunosuppression
5.7 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.
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