Jeremy B. Levy, MA, PhD, MRCP; A. Neil Turner, PhD, FRCP; Andrew J. Rees, MSc, FRCP, FMedSci; Charles D. Pusey, MSc, FRCP, FRCPath
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.
Levy JB, Turner AN, Rees AJ, Pusey CD. Long-Term Outcome of Anti–Glomerular Basement Membrane Antibody Disease Treated with Plasma Exchange and Immunosuppression. Ann Intern Med. 2001;134:1033-1042. doi: 10.7326/0003-4819-134-11-200106050-00009
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Published: Ann Intern Med. 2001;134(11):1033-1042.
Anti–glomerular basement membrane (GBM) antibody disease is a rare autoimmune disorder characterized by rapidly progressive glomerulonephritis with crescentic changes that affect most glomeruli on renal biopsy; when accompanied by pulmonary hemorrhage, the condition is known as the Goodpasture syndrome (1, 2). The disease is by definition associated with development of anti-GBM autoantibodies. These antibodies have proven pathogenicity (3) and bind the noncollagenous domain (NC1) of the α3 chain of type IV collagen (4-6).
Historically, untreated patients do not recover renal function and have substantial mortality, particularly from pulmonary hemorrhage (1). The introduction of oral immunosuppression did not obviously alter the prognosis of the disease; however, the use of plasma exchange in combination with prednisolone and cyclophosphamide dramatically improved outcomes (7, 8). Data on short-term renal and patient survival from most centers show that 75% to 95% of patients live for at least 1 year after presentation but no more than 40% of patients will have independent renal function at 1 year, depending on the degree of renal failure at presentation (1). Most studies have shown a clear association between the extent of glomerular crescent formation and renal outcome (9, 10), and some show a loose correlation between antibody titer and disease severity (11).
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