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Clinical and Experimental Nephrology
Published in: Clinical and Experimental Nephrology 4/2017

Open Access 01-08-2017 | Review article

C3 glomerulopathy and current dilemmas

Authors: Naoko Ito, Ryuji Ohashi, Michio Nagata

Published in: Clinical and Experimental Nephrology | Issue 4/2017

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Abstract

C3 glomerulopathy (C3G) is a recently identified disease entity caused by dysregulation of the alternative complement pathway, and dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) are its components. Because laboratory detection of complement dysregulation is still uncommon in practice, “dominant C3 deposition by two orders greater than that of immunoglobulins in the glomeruli by immunofluorescence”, as stated in the consensus report, defines C3G. However, this morphological definition possibly includes the cases with glomerular diseases of different mechanisms such as post-infectious glomerulonephritis. In addition, the differential diagnosis between DDD and C3GN is often difficult because the distinction between these two diseases is based solely on electron microscopic features. Recent molecular and genetic advances provide information to characterize C3G. Some C3G cases are found with genetic abnormalities in complement regulatory factors, but majority of cases seem to be associated with acquired factors that dysregulate the alternative complement pathway. Because clinical courses and prognoses among glomerular diseases with dominant C3 deposition differ, further understanding the background mechanism, particularly complement dysregulation in C3G, is needed. This may resolve current dilemmas in practice and shed light on novel targeted therapies to remedy the dysregulated alternative complement pathway in C3G.
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Metadata
Title
C3 glomerulopathy and current dilemmas
Authors
Naoko Ito
Ryuji Ohashi
Michio Nagata
Publication date
01-08-2017
Publisher
Springer Japan
Published in
Clinical and Experimental Nephrology / Issue 4/2017
Print ISSN: 1342-1751
Electronic ISSN: 1437-7799
DOI
https://doi.org/10.1007/s10157-016-1358-5

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