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Complement C5-inhibiting therapy for the thrombotic microangiopathies: accumulating evidence, but not a panacea

Lookup NU author(s): Dr Vicky Brocklebank, Professor David Kavanagh

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This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC 4.0).


Abstract

Thrombotic microangiopathy (TMA), characterized by organ injury occurring consequent to severe endothelial damage, can manifest in a diverse range of diseases. In complement mediated atypical haemolytic uraemic syndrome (aHUS) a primary defect in complement, such as a mutation or autoantibody leading to over activation of the alternative pathway, predisposes to the development of disease, usually following exposure to an environmental trigger. The elucidation of the pathogenesis of aHUS resulted in the successful introduction of the complement inhibitor eculizumab into clinical practice. In other TMAs, although complement activation may be seen, its role in the pathogenesis remains to be confirmed by an interventional trial. Although many case reports in TMAs other than complement mediated aHUS hint at efficacy, publication bias, concurrent therapies and in some cases the self-limiting nature of disease make broader interpretation difficult. In this article we will review the evidence for the role of complement inhibition in complement mediated aHUS and other TMAs.


Publication metadata

Author(s): Brocklebank V, Kavanagh D

Publication type: Article

Publication status: Published

Journal: Clinical Kidney Journal

Year: 2017

Volume: 10

Issue: 5

Pages: 600-624

Print publication date: 01/10/2017

Online publication date: 08/05/2017

Acceptance date: 29/09/2017

ISSN (print): 2048-8505

ISSN (electronic): 2048-8513

Publisher: Oxford University Press

URL: https://doi.org/10.1093/ckj/sfx081

DOI: 10.1093/ckj/sfx081

Notes: https://academic.oup.com/ckj/article/10/5/600/4283135/Complement-C5-inhibiting-therapy-for-the


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