IgA vasculitis and anti-GBM disease: two ends of a spectrum of immune complex vasculitis. (29th April 2020)

• Record Type: Journal Article
• Authors: Salama, Alan D
• Is Part Of: Rheumatology. Volume 59(2020)Supplement 3
• Page Start: iii118
• Page End: iii122
• Publication Date: 2020-04-29
• Subjects: anti-GBM disease -- Goodpasture's disease -- Henoch–Schönlein purpura -- IgA vasculitis -- pathogenesis -- outcomes -- vasculitis -- crescentic glomerulonephritis; Rheumatism -- Periodicals; Rheumatology -- Periodicals; 616.723005
• DOI: 10.1093/rheumatology/kez540
• Languages: English
• ISSNs: 1462-0324

Abstract

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.