C3 Glomerulopathy

Jill J Hauer; Carla M Nester; Richard J. H Smith

doi:10.1007/978-3-319-49379-4_41

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Book chapter

C3 Glomerulopathy

Jill J Hauer, Carla M Nester and Richard J. H Smith

Glomerulonephritis, pp.633-646

Springer International Publishing

02/27/2019

DOI: 10.1007/978-3-319-49379-4_41

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Abstract

C3 Glomerulopathy (C3G) defines a group of complement-mediated renal diseases that share specific features identifiable on kidney biopsy the hallmark of which is C3 dominance on immunofluorescence by at least two orders of magnitude greater than any other immuno-reactant; electron microscopy is also specific. These features should prompt a thorough evaluation of the complement system that covers four domains: 1) genetic drivers of disease; 2) acquired drivers of disease; 3) biomarker profiling; and, 4) tests of complement function. The aggregate picture afforded by these studies provides insight into disease status and can guide treatment. Although optimal disease-directed treatment has not be determined, most patients should receive angiotensin‐converting enzyme inhibitors or angiotensin II receptor blocker for their anti-proteinuric and nephro-protective effects. The value of anti-cellular immune suppression to target T and/or B cells is controversial. The role of complement abnormalities has focused attention on anti-complement therapies as potential treatments and it is well documented that some, but not all, patients respond to eculizumab with elevation of soluble C5b-9 being a potentially useful marker of a responder to this terminal pathway blocker. No specific data are available to inform decisions surrounding transplantation. The development of novel complement inhibitors is a high priority for these patients.

alternative pathway

C3 glomerulonephritis

C3 glomerulopathy

Complement dysregulation

Dense deposit disease

Details

Title: Subtitle: C3 Glomerulopathy
Creators: Jill J Hauer - Molecular Otolaryngology and Renal Research Laboratories (MORL), University of Iowa, Iowa City, USA
Carla M Nester - Rare Renal Diseases Clinic, University of Iowa, Iowa City, USA
Richard J. H Smith - Rare Renal Diseases Clinic, University of Iowa, Iowa City, USA
Resource Type: Book chapter
Publication Details: Glomerulonephritis, pp.633-646
DOI: 10.1007/978-3-319-49379-4_41
Publisher: Springer International Publishing; Cham
Language: English
Date published: 02/27/2019
Academic Unit: Roy J. Carver Department of Biomedical Engineering; Molecular Physiology and Biophysics; Anatomy and Cell Biology; Nephrology, Dialysis and Transplantation; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Otolaryngology; Internal Medicine
Record Identifier: 9984256929402771

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