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Seminars in Immunopathology
Published in: Seminars in Immunopathology 1/2018

Open Access 01-01-2018 | Review

Diseases of complement dysregulation—an overview

Authors: Edwin K. S. Wong, David Kavanagh

Published in: Seminars in Immunopathology | Issue 1/2018

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Abstract

Atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy (C3G), and paroxysmal nocturnal hemoglobinuria (PNH) are prototypical disorders of complement dysregulation. Although complement overactivation is common to all, cell surface alternative pathway dysregulation (aHUS), fluid phase alternative pathway dysregulation (C3G), or terminal pathway dysregulation (PNH) predominates resulting in the very different phenotypes seen in these diseases. The mechanism underlying the dysregulation also varies with predominant acquired autoimmune (C3G), somatic mutations (PNH), or inherited germline mutations (aHUS) predisposing to disease. Eculizumab has revolutionized the treatment of PNH and aHUS although has been less successful in C3G. With the next generation of complement therapeutic in late stage development, these archetypal complement diseases will provide the initial targets.
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Metadata
Title
Diseases of complement dysregulation—an overview
Authors
Edwin K. S. Wong
David Kavanagh
Publication date
01-01-2018
Publisher
Springer Berlin Heidelberg
Published in
Seminars in Immunopathology / Issue 1/2018
Print ISSN: 1863-2297
Electronic ISSN: 1863-2300
DOI
https://doi.org/10.1007/s00281-017-0663-8

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