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Binding Truths: Atypical anti-GBM disease mediated by IgA anti-GBM antibodies targeting the α1 chain of type IV collagen

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. Green open access

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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.

Type: Article
Title: Binding Truths: Atypical anti-GBM disease mediated by IgA anti-GBM antibodies targeting the α1 chain of type IV collagen
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.ekir.2018.08.005
Publisher version: https://doi.org/10.1016/j.ekir.2018.08.005
Language: English
Additional information: This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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/10054450
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