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C3 glomerulopathy: Understanding an ultra-rare complement-mediated renal disease
Journal article   Open access   Peer reviewed

C3 glomerulopathy: Understanding an ultra-rare complement-mediated renal disease

Amanda K Heiderscheit, Jill J Hauer and Richard J H Smith
American journal of medical genetics. Part C, Seminars in medical genetics, Vol.190(3), pp.344-357
06/23/2022
DOI: 10.1002/ajmg.c.31986
PMCID: PMC9613507
PMID: 35734939
Appears in  UI Libraries Support Open Access
url
https://doi.org/10.1002/ajmg.c.31986View
Published (Version of record) Open Access

Abstract

C3 glomerulopathy (C3G) describes a pathologic pattern of injury diagnosed by renal biopsy. It is characterized by the dominant deposition of the third component of complement (C3) in the renal glomerulus as resolved by immunofluorescence microscopy. The underlying pathophysiology is driven by dysregulation of the alternative pathway of complement in the fluid-phase and in the glomerular microenvironment. Characterization of clinical features and a targeted evaluation for indices and drivers of complement dysregulation are necessary for optimal patient care. Autoantibodies to the C3 and C5 convertases of complement are the most commonly detected drivers of complement dysregulation, although genetic mutations in complement genes can also be found. Approximately half of patients progress to end-stage renal disease within 10 years of diagnosis, and, while transplantation is a viable option, there is high risk for disease recurrence and allograft failure. This poor outcome reflects the lack of disease-specific therapy for C3G, relegating patients to symptomatic treatment to minimize proteinuria and suppress renal inflammation. Fortunately, the future is bright as several anti-complement drugs are currently in clinical trials.
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