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Diagnostic and management challenges in Goodpasture's (anti-glomerular basement membrane) disease

Henderson, S; Salama, A; (2018) Diagnostic and management challenges in Goodpasture's (anti-glomerular basement membrane) disease. Nephrology, Dialysis and Transplantation , 33 (2) pp. 196-202. 10.1093/ndt/gfx057. Green open access

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Abstract

Goodpasture’s or anti-glomerular basement membrane (GBM) disease is classically characterized by the presence of circulating autoantibodies directed against the non-collagenous domain of the α3 chain of type IV collagen, targeting glomerular and alveolar basement membranes, and associated with rapidly progressive crescentic glomerulonephritis, with alveolar haemorrhage in over half the patients. However, there are increasing examples of variants or atypical presentations of this disease, and novel therapeutic options have been proposed, which nephrologists should be aware of. The pathophysiology of this condition has been understood through molecular analysis of the antibody–antigen interactions and the use of human leucocyte antigen-transgenic animals, while the association of anti-GBM antibodies with anti-neutrophil cytoplasm antibodies and their combined impact on disease phenotype is increasingly recognized, providing some insights into the basis of glomerular damage and autoimmunity.

Type: Article
Title: Diagnostic and management challenges in Goodpasture's (anti-glomerular basement membrane) disease
Open access status: An open access version is available from UCL Discovery
DOI: 10.1093/ndt/gfx057
Publisher version: https://doi.org/10.1093/ndt/gfx057
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: AKI, anti-GBM, glomerulonephritis, Goodpasture's
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Renal Medicine
URI: https://discovery.ucl.ac.uk/id/eprint/1544922
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