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Rheum Dis Clin North Am. 2018 Nov;44(4):651-673. doi: 10.1016/j.rdc.2018.06.011. Epub 2018 Sep 7.

Anti-Glomerular Basement Membrane Disease.

Author information

1
Renal and Vascular Inflammation Section, Department of Medicine, Imperial College London, Commonwealth Building, Hammersmith Hospital Campus, Du Cane Road, London W12 ONN, UK; Vasculitis Clinic, Imperial College Healthcare NHS Trust, Hammersmith Hospital, Du Cane Road, London W12 0HS, UK.
2
Renal and Vascular Inflammation Section, Department of Medicine, Imperial College London, Commonwealth Building, Hammersmith Hospital Campus, Du Cane Road, London W12 ONN, UK; Vasculitis Clinic, Imperial College Healthcare NHS Trust, Hammersmith Hospital, Du Cane Road, London W12 0HS, UK. Electronic address: s.mcadoo@imperial.ac.uk.

Abstract

Anti-glomerular basement membrane (anti-GBM) disease is a rare autoimmune small vessel vasculitis characterized by autoreactivity to antigens in type IV collagen chains expressed in glomerular and alveolar basement membrane. The detection of circulating anti-GBM antibodies, which are shown to be directly pathogenic, is central to disease diagnosis. Clinically, anti-GBM disease usually presents with rapidly progressive glomerulonephritis with or without alveolar hemorrhage. Rapid diagnosis and early treatment are required to prevent mortality and to preserve renal function. Relapse in anti-GBM disease is uncommon. Variant and atypical forms of anti-GBM disease are increasingly recognised.

KEYWORDS:

Goodpasture syndrome; Plasma exchange; Pulmonary hemorrhage; Rapidly progressive glomerulonephritis; Vasculitis

PMID:
30274629
DOI:
10.1016/j.rdc.2018.06.011
[Indexed for MEDLINE]

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