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Complement therapy in atypical haemolytic uraemic syndrome (aHUS)

Lookup NU author(s): Dr Edwin Wong, Professor Tim Goodship, Professor David KavanaghORCiD



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


Central to the pathogenesis of atypical haemolytic uraemic syndrome (aHUS) is over-activation of the alternative pathway of complement. Inherited defects in complement genes and autoantibodies against complement regulatory proteins have been described. The use of plasma exchange to replace non-functioning complement regulators and hyper-functional complement components in addition to the removal of CFH-autoantibodies made this the 'gold-standard' for management of aHUS. In the last 4 years the introduction of the complement inhibitor Eculizumab has revolutionised the management of aHUS. In this review we shall discuss the available literature on treatment strategies to date. (C) 2013 Elsevier Ltd. All rights reserved.

Publication metadata

Author(s): Wong EKS, Goodship THJ, Kavanagh D

Publication type: Review

Publication status: Published

Journal: Molecular Immunology

Year: 2013

Volume: 56

Issue: 3

Pages: 199-212

Print publication date: 28/06/2013

Acceptance date: 10/05/2013

ISSN (print): 0161-5890

ISSN (electronic): 1872-9142



DOI: 10.1016/j.molimm.2013.05.224