Chapter

Antiglomerular basement membrane disease

Jeremy Levy and Charles Pusey

in Oxford Textbook of Medicine

Fifth edition

Published on behalf of Oxford University Press

ISBN: 9780199204854
Published online May 2012 | e-ISBN: 9780199570973 | DOI: http://dx.doi.org/10.1093/med/9780199204854.003.210807_update_001

Series: Oxford Textbooks

Antiglomerular basement membrane disease

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Antiglomerular basement membrane disease (anti-GBM disease, also known as Goodpasture’s disease) is a rare autoimmune disease caused by pathogenic autoantibodies directed against the noncollagenous, C-terminal domain of the α-3 chain of type IV collagen (α3(IV)NC1). Immunohistology is characteristic, with linear deposition of IgG (sometimes with IgA or IgM) and complement C3 along the GBMs.

Clinical features—anti-GBM disease classically presents with pulmonary haemorrhage (in two-thirds of patients) and rapidly progressive glomerulonephritis (RPGN, with haematuria and proteinuria, urinary red cell casts, and typically severe acute renal failure). Haemoptysis can be triggered by cigarettes, inhaled toxins, fluid overload, and intercurrent infection. The condition must be distinguished from other cause of RPGN, especially antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, in particular because there is only a small window of opportunity in which to rescue renal function in patients with Goodpasture’s disease. Key investigations are (for diagnosis) serological testing for anti-GBM antibodies and ANCA, and renal biopsy, and (for detection of pulmonary haemorrhage) chest radiology and estimation of carbon monoxide transfer factor (Kco).

Management and prognosis—untreated anti-GBM disease is usually fatal, and renal function never recovers. Immunosuppressive treatment (plasma exchange, cyclophosphamide, oral steroids) is given immediately on diagnosis to patients with serum creatinine levels lower than 600 µmol/litre at presentation and/or with active pulmonary haemorrhage. In contrast, patients with serum creatinine levels more than 600 µmol/litre at presentation rarely recover renal function, hence immunosuppressive treatment in such cases would be restricted in most centres to those with pulmonary haemorrhage or in whom the renal biopsy suggested additional mechanisms of renal damage such as acute tubular necrosis. In recent series, 1-year patient survival is 66 to 92%, 1-year renal survival is 15 to 59%, with renal recovery in patients with initial creatinine levels greater than 600 µmol/litre of 0 to 21%. Patients do not need long-term immunosuppression, and the disease rarely recurs. Transplantation is safe if performed after autoantibodies have been suppressed or naturally disappeared.

Chapter.  3963 words.  Illustrated.

Subjects: Nephrology

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