title: Complement inhibition in C3 glomerulopathy.
abstract: C3 glomerulopathy (C3G) describes a spectrum of glomerular diseases defined by shared renal biopsy pathology: a predominance of C3 deposition on immunofluorescence with electron microscopy permitting disease sub-classification. Complement dysregulation underlies the observed pathology, a causal relationship that is supported by well described studies of genetic and acquired drivers of disease. In this article, we provide an overview of the features of C3G, including a discussion of disease definition and a review of the causal role of complement. We discuss molecular markers of disease and how biomarkers are informing our evolving understanding of underlying pathology. Research advances are laying the foundation for complement inhibition as a targeted approach to treatment of C3G.
authors.affiliation: Molecular Otolaryngology and Renal Research Laboratories, Carver College of Medicine, University of Iowa, Iowa City, IA, USA; Divisions of Nephrology, Stead Family Department of Pediatrics, Department of Internal Medicine, Carver College of Medicine, University of Iowa, Iowa City, IA, USA.
authors.forename: Carla M
authors.initials: CM
authors.lastname: Nester
authors.affiliation: Molecular Otolaryngology and Renal Research Laboratories, Carver College of Medicine, University of Iowa, Iowa City, IA, USA; Divisions of Nephrology, Stead Family Department of Pediatrics, Department of Internal Medicine, Carver College of Medicine, University of Iowa, Iowa City, IA, USA. Electronic address: richard-smith@uiowa.edu.
authors.forename: Richard J H
authors.initials: RJ
authors.lastname: Smith
mesh_terms: D000818:Animals; D015415:Biomarkers / Q000378:metabolism*; D003176:Complement C3 / Q000235:genetics / Q000276:immunology*; D051056:Complement Inactivating Agents / Q000627:therapeutic use*; D003170:Complement Pathway, Alternative; D005787:Gene Frequency; D005838:Genotype; D015433:Glomerulonephritis, Membranous / Q000235:genetics / Q000276:immunology / Q000628:therapy*; D006801:Humans; D007167:Immunotherapy / Q000379:methods*; D007668:Kidney / Q000378:metabolism* / Q000473:pathology; D011110:Polymorphism, Genetic
chemical_list: D015415:Biomarkers; D003176:Complement C3; D051056:Complement Inactivating Agents
keywords: C3 glomerulonephritis; C3 glomerulopathy; Complement; Dense deposit disease; Eculizumab; Membranoproliferative glomerulonephritis