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IgA Vasculitis and Anti-GBM disease: two ends of a spectrum of immune complex vasculitis

Salama, A; (2020) IgA Vasculitis and Anti-GBM disease: two ends of a spectrum of immune complex vasculitis. Rheumatology , 59 (S3) iii118-iii122. 10.1093/rheumatology/kez540. Green open access

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Abstract

Two immune complex vasculitides, IgA vasculitis (IgAV) and anti-GBM disease, represent polar extremes with regard to our understanding of disease pathogenesis, standardized management protocols and outcomes. This report compares our current approach to these uncommon entities in adults. Both diseases demonstrate degrees of small vessel necrosis and glomerular crescent formation. IgAV has an antibody response directed against unknown antigens, is often treated conservatively and has poorly studied long term renal outcomes. By contrast, anti-GBM disease presents with rapidly progressive glomerulonephritis and often results in end stage renal failure, despite intensive immunosuppression. Rarely, some cases of anti-GBM disease may be IgA predominant and bind other α-chains present in the GBM, but their clinical course is as for other anti-GBM disease patients but not IgAV, suggesting that the antigenic target rather than the antibody subclass is the critical factor in determining disease outcome. However, both conditions are associated with increased mortality in adults and result in significant chronic kidney disease and hypertension.

Type: Article
Title: IgA Vasculitis and Anti-GBM disease: two ends of a spectrum of immune complex vasculitis
Open access status: An open access version is available from UCL Discovery
DOI: 10.1093/rheumatology/kez540
Publisher version: https://doi.org/10.1093/rheumatology/kez540
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: anti-GBM disease, Goodpasture’s disease, Henoch–Schönlein purpura, IgA vasculitis, pathogenesis, outcomes, vasculitis, crescentic glomerulonephritis
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/10084680
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