title: [C3 glomerulopathy].
abstract: C3 glomerulopathy is an heterogeneous group of glomerular diseases associated with acquired or genetic abnormalities of complement alternative pathway (AP) components. It is characterized by predominant C3 deposits in the mesangium and along the glomerular basement membrane (GBM). Presenting features comprise proteinuria (sometimes with nephritic syndrome), haematuria, hypertension and renal failure. C3 glomerulopathy have a poor renal prognosis with progression to end stage renal disease (ESRD) in 50% of cases during the first decade after initial presentation. Moreover, C3 deposits recur in most of cases after renal transplantation. Patients frequently have low serum C3 level attributed to the activation of the alternative pathway of complement. Animal models have confirmed the role of excessive C3 activation in the pathogenesis of C3 glomerulopathy. To date, the optimal treatment remains unknown. It is currently based on the use of angiotensin-converting-enzyme inhibitors (ACEI) and angiotensin II-receptor blockers (ARB), sometimes associated with immunosuppressive therapy. Blockade of C5a release with eculizumab, a monoclonal anti-C5 antibody, may be of particular interest in the treatment of C3G.
authors: Service de néphrologie, hôpital Georges-Pompidou, 20, rue Leblanc, 75015 Paris, France. Electronic address: sophie.chauvet@egp.aphp.fr.
authors: Sophie
authors: S
authors: Chauvet
authors: Service de néphrologie, hôpital Necker-Enfants-Malades, 149, rue de Sèvres, 75015 Paris, France.
authors: Aude
authors: A
authors: Servais
authors: Laboratoire d'immunologie, hôpital Georges-Pompidou, 20, rue Leblanc, 75015 Paris, France.
authors: Véronique
authors: V
authors: Frémeaux-Bacchi
mesh_terms: D015415:Biomarkers / Q000097:blood; D003176:Complement C3 / Q000378:metabolism*; D018450:Disease Progression; D019317:Evidence-Based Medicine; D050533:Glomerular Basement Membrane / Q000473:pathology; D005920:Glomerular Mesangium / Q000473:pathology; D015432:Glomerulonephritis, Membranoproliferative / Q000097:blood / Q000276:immunology* / Q000473:pathology* / Q000628:therapy; D006417:Hematuria / Q000276:immunology; D006801:Humans; D006973:Hypertension / Q000276:immunology; D007155:Immunologic Factors / Q000378:metabolism; D007676:Kidney Failure, Chronic / Q000276:immunology; D011379:Prognosis; D011507:Proteinuria / Q000276:immunology
chemical_list: D015415:Biomarkers; D003176:Complement C3; D007155:Immunologic Factors
keywords: Activation de la voie alterne; Complement alternative pathway; Complément; Glomerulopathy; Glomérulopathie à C3