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Review
. 2017 Mar 10:6:248.
doi: 10.12688/f1000research.10364.1. eCollection 2017.

C3 glomerulopathy

H Terence Cook  1
Affiliations
Review

C3 glomerulopathy

H Terence Cook. F1000Res. .

Abstract

C3 glomerulopathy is a recently defined entity that encompasses a group of kidney diseases caused by abnormal control of complement activation with deposition of complement component C3 in glomeruli leading to variable glomerular inflammation. Before the recognition of the unique pathogenesis of these cases, they were variably classified according to their morphological features. C3 glomerulopathy accounts for roughly 1% of all renal biopsies. Clear definition of this entity has allowed a better understanding of its pathogenesis and clinical course and is likely to lead to the design of rational therapies over the next few years.

Keywords: C3 complement; glomerular inflammation; renal pathology.

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Conflict of interest statement

Competing interests: The author declares that he has consulting agreements with Alexion Pharmaceuticals and Achillion Pharmaceuticals.Competing interests: J.D.L is an inventor of patents and patent application describing complement inhibitors and their clinical use. He is also the founder of Amyndas Pharmaceuticals, which is developing complement inhibitors for therapeutic purposes.No competing interests were disclosed.No competing interests were disclosed.No competing interests were disclosed.No competing interests were disclosed.

Figures

Figure 1.
Figure 1.. C3 staining in C3 glomerulopathy.
Glomerulus showing staining for C3 in a case of C3 glomerulopathy. The kidney is stained with a fluorescently labelled antibody to C3.
Figure 2.
Figure 2.. Electron micrograph showing dense transformation of the glomerular basement membrane in a case of dense deposit disease.
Figure 3.
Figure 3.. Electron micrograph showing multiple electron-dense deposits in the mesangium and capillary wall of a glomerulus in a case of C3 glomerulonephritis.
Figure 4.
Figure 4.. Glomerulus from a case of C3 glomerulonephritis showing a membranoproliferative glomerulonephritis pattern.
There is increased mesangial matrix and mesangial cells. Capillary walls are thickened with segmental double contours.
See this image and copyright information in PMC

References

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    2. F1000 Recommendation

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