title: C3 glomerulopathy: A new complement-based entity.
abstract: C3 glomerulopathy is a new, recently described entity that has changed the perspective, treatment and classification of a number of glomerular diseases. It encompasses 2 similar but clearly differentiated pathologies -the dense-deposit disease and C3 glomerulonephritis itself. The alternative complement pathway plays a fundamental role in its pathogenesis and, specifically, the mutations and defects in its regulatory factors (mainly factor H and factor I), as well as the presence of acquired autoantibodies (C3 nephritic factor), which generates an unbridled activation of the system, and ultimately, a deposit of its products at the glomerular level. Its poor prognosis and onset in young populations makes the detailed study of new therapeutic alternatives for this disease essential. Recently eculizumab, an anti-C5 antibody, has demonstrated effectiveness in the treatment of these patients.
authors.affiliation: Servicio de Nefrología, Hospital Universitario de Guadalajara, Departamento de Medicina, Universidad de Alcalá, Alcalá de Henares, Madrid, España. Electronic address: doctorberto@hotmail.com.
authors.forename: A
authors.initials: A
authors.lastname: de Lorenzo
authors.affiliation: Servicio de Nefrología, Hospital Universitario de Guadalajara, Departamento de Medicina, Universidad de Alcalá, Alcalá de Henares, Madrid, España.
authors.forename: S
authors.initials: S
authors.lastname: Tallón
authors.affiliation: Servicio de Nefrología, Hospital Universitario de Guadalajara, Departamento de Medicina, Universidad de Alcalá, Alcalá de Henares, Madrid, España.
authors.forename: B
authors.initials: B
authors.lastname: Hernández-Sevillano
authors.affiliation: Servicio de Nefrología, Hospital Universitario de Guadalajara, Departamento de Medicina, Universidad de Alcalá, Alcalá de Henares, Madrid, España.
authors.forename: G
authors.initials: G
authors.lastname: de Arriba
mesh_terms: D061067:Antibodies, Monoclonal, Humanized / Q000494:pharmacology / Q000627:therapeutic use; D001323:Autoantibodies / Q000276:immunology*; D003176:Complement C3 / Q000276:immunology; D003178:Complement C3 Nephritic Factor / Q000276:immunology*; D003182:Complement C5 / Q000037:antagonists & inhibitors; D003170:Complement Pathway, Alternative / Q000276:immunology; D005921:Glomerulonephritis / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology; D015432:Glomerulonephritis, Membranoproliferative / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology*; D006801:Humans; D011379:Prognosis
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D001323:Autoantibodies; D003176:Complement C3; D003178:Complement C3 Nephritic Factor; D003182:Complement C5; C481642:eculizumab
keywords: Alternative complement pathway; C3 glomerulopathy; C3 nephritic factor; Dense-deposit disease; Eculizumab; Enfermedad por depósitos densos; Factor H; Factor I; Factor nefrítico C3; Glomerulopatía C3; Vía alternativa del complemento