title: Update on C3 glomerulopathy.
abstract: C3 glomerulopathy refers to a disease process in which abnormal control of complement activation, degradation or deposition results in predominant C3 fragment deposition within the glomerulus and glomerular damage. Recent studies have improved our understanding of its pathogenesis. The key abnormality is uncontrolled C3b amplification in the circulation and/or along the glomerular basement membrane. Family studies in which disease segregates with structurally abnormal complement factor H-related (CFHR) proteins demonstrate that abnormal CFHR proteins are important in some types of C3 glomerulopathy. This is currently thought to be due to the ability of these proteins to antagonize the major negative regulator of C3 activation, complement factor H (CFH), a process termed 'CFH de-regulation'. Recent clinicopathological cohort studies have led to further refinements in case definition, culminating in a 2013 consensus report, which provides recommendations regarding investigation and treatment. Early clinical experience with complement-targeted therapeutics, notably C5 inhibitors, has also now been published. Here, we summarize the latest developments in C3 glomerulopathy.
authors.affiliation: Centre for Complement and Inflammation Research (CCIR), Division of Immunology and Inflammation, Department of Medicine, Imperial College London, London W12 0NN, UK.
authors.forename: Thomas D
authors.initials: TD
authors.lastname: Barbour
authors.affiliation: Centre for Complement and Inflammation Research (CCIR), Division of Immunology and Inflammation, Department of Medicine, Imperial College London, London W12 0NN, UK.
authors.forename: Marieta M
authors.initials: MM
authors.lastname: Ruseva
authors.affiliation: Centre for Complement and Inflammation Research (CCIR), Division of Immunology and Inflammation, Department of Medicine, Imperial College London, London W12 0NN, UK.
authors.forename: Matthew C
authors.initials: MC
authors.lastname: Pickering
mesh_terms: D000818:Animals; D003167:Complement Activation; D003176:Complement C3 / Q000378:metabolism*; D017242:Complement Factor H / Q000378:metabolism*; D006801:Humans; D007674:Kidney Diseases / Q000503:physiopathology*; D007678:Kidney Glomerulus / Q000378:metabolism / Q000473:pathology*
chemical_list: D003176:Complement C3; D017242:Complement Factor H
keywords: C3 glomerulopathy; complement; dense deposit; factor H