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Atypical aHUS: State of the art

Lookup NU author(s): Dr Veronique Fremaux-Bacchi, Professor Tim Goodship, Professor David KavanaghORCiD

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Abstract

Tremendous advances in our understanding of the thrombotic microangiopathies (TMAs) have revealed distinct disease mechanisms within this heterogeneous group of diseases. As a direct result of this knowledge, both children and adults with complement-mediated TMA now enjoy higher expectations for long-term health. In this update on atypical hemolytic uremic syndrome, we review the clinical characteristics; the genetic and acquired drivers of disease; the broad spectrum of environmental triggers; and current diagnosis and treatment options. Many questions remain to be addressed if additional improvements in patient care and outcome are to be achieved in the coming decade. (C) 2015 Elsevier Ltd. All rights reserved.

Publication metadata

Author(s): Nester CM, Barbour T, de Cordoba SR, Dragon-Durey MA, Fremeaux-Bacchi V, Goodship THJ, Kavanagh D, Noris M, Pickering M, Sanchez-Corral P, Skerka C, Zipfel P, Smith RJH

Publication type: Review

Publication status: Published

Journal: Molecular Immunology

Year: 2015

Volume: 67

Issue: 1

Pages: 31-42

Print publication date: 01/09/2015

Online publication date: 03/04/2015

Acceptance date: 12/03/2015

ISSN (print): 0161-5890

ISSN (electronic): 1872-9142

Publisher: PERGAMON-ELSEVIER SCIENCE LTD

URL: http://dx.doi.org/10.1016/j.molimm.2015.03.246

DOI: 10.1016/j.molimm.2015.03.246

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