Elsevier

Molecular Immunology

Volume 56, Issue 3, 15 December 2013, Pages 199-212
Molecular Immunology

Review
Complement therapy in atypical haemolytic uraemic syndrome (aHUS)

https://doi.org/10.1016/j.molimm.2013.05.224Get rights and content
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Abstract

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.

Keywords

Eculizumab
Haemolytic Uraemic Syndrome
Treatment
Complement

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