Antonelou, M; Henderson, SR; Bhangal, G; Oliveira, B; Hamour, S; HARBER, M; Salama, AD; (2019) Binding Truths: Atypical anti-GBM disease mediated by IgA anti-GBM antibodies targeting the α1 chain of type IV collagen. Kidney International Reports , 4 (1) pp. 163-167. 10.1016/j.ekir.2018.08.005.

Preview

Text Antonelou VoR 1-s2.0-S2468024918301906-main.pdf - Published Version Download (1MB) | Preview

Abstract

Anti−glomerular basement membrane (anti-GBM) disease presents with rapidly progressive glomerulonephritis, often associated with alveolar hemorrhage and characterized histologically by crescentic glomerulonephritis. Typically, there is linear deposition of Ig along the glomerular basement membrane (GBM), which, in the majority of cases, is due to IgG autoantibodies directed against the noncollagenous domain of the α3 chain of type IV collagen (α3[IV]NC1).1 Early disease recognition relies on detecting circulating IgG anti-GBM antibodies in serum samples. However, conventional assays do not detect IgA antibodies or those directed against other target antigens, including α5(IV) found in some Alport disease patients following renal transplantation.2 The presence and specificity of the antibody can be confirmed by Western blotting, usually at a reference center, although this is not routinely performed. We describe a case of anti-GBM disease mediated by IgA anti-GBM antibodies not detected by standard serological tests, and suggest a method of detection and monitoring that can be used in the right clinical context.

Download activity - last month

Export as CSVXMLJSON

Download activity - last 12 months

Export as JSONXMLCSV

Downloads by country - last 12 months

Export as CSVXMLJSON

Archive Staff Only

View Item