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Clinical and Developmental Immunology
Volume 2012 (2012), Article ID 695131, 6 pages
http://dx.doi.org/10.1155/2012/695131
Review Article

Kidney Diseases Caused by Complement Dysregulation: Acquired, Inherited, and Still More to Come

1Division of Internal Medicine, University Hospital Zurich, Raemistrasse 100, 8006 Zurich, Switzerland
2Division of Nephrology, University Hospital Zurich, Raemistrasse 100, 8006 Zurich, Switzerland

Received 6 August 2012; Accepted 11 October 2012

Academic Editor: Michael A. Flierl

Copyright © 2012 Saskia F. Heeringa and Clemens D. Cohen. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Inherited and acquired dysregulation of the complement alternative pathway plays an important role in multiple renal diseases. In recent years, the identification of disease-causing mutations and genetic variants in complement regulatory proteins has contributed significantly to our knowledge of the pathogenesis of complement associated glomerulopathies. In these diseases defective complement control leading to the deposition of activated complement products plays a key role. Consequently, complement-related glomerulopathies characterized by glomerular complement component 3 (C3) deposition in the absence of local immunoglobulin deposits are now collectively described by the term “C3 glomerulopathies.” Therapeutic strategies for reestablishing complement regulation by either complement blockade with the anti-C5 monoclonal antibody eculizumab or plasma substitution have been successful in several cases of C3 glomerulopathies. However, further elucidation of the underlying defects in the alternative complement pathway is awaited to develop pathogenesis-specific therapies.