title: [The complement system in the pathogenesis of antineutrophil cytoplasm antibodies-associated vasculitis].
abstract: One of the main characteristics of the vasculitis associated with antineutrophil cytoplasm autoantibodies (AASV) is the absence of immune complex deposition in biopsies of affected tissues as well as a lack of complement depletion. However, in early stages of disease induced in animal models, it has been observed that the complement system may be involved in the generation of these diseases. There are various animal models which have been developed with the aim of knowing which are the pathogenic mechanisms in granulomatosis with polyangiitis (Wegener) (GPA) and microscopic polyangiitis (MPA), the latter being explained using these approaches in a more satisfactory manner, as there is lack of a model which reproduces the changes leading to a granulomatous vasculitis associated with antibodies against proteinase-3, as in GPA. This short review presents recent evidence of the presence of complement in biopsies of patients with AASV and the most recent animal models, which show the participation of complement in their etiology.
authors.affiliation: Clínica de Vasculitis Sistémicas Primarias, Instituto Nacional de Enfermedades Respiratorias, México DF, México. felipe98@Prodigy.Net.Mx
authors.forename: Luis F
authors.initials: LF
authors.lastname: Flores-Suárez
mesh_terms: D000818:Animals; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000276:immunology*; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis / Q000276:immunology*; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D001324:Autoantigens / Q000276:immunology; D001706:Biopsy; D003167:Complement Activation; D003182:Complement C5 / Q000037:antagonists & inhibitors; D015936:Complement C5a / Q000037:antagonists & inhibitors; D003165:Complement System Proteins / Q000276:immunology*; D004268:DNA-Binding Proteins / Q000172:deficiency; D014890:Granulomatosis with Polyangiitis / Q000276:immunology; D006801:Humans; D007668:Kidney / Q000276:immunology / Q000473:pathology; D051379:Mice; D008810:Mice, Inbred C57BL; D055953:Microscopic Polyangiitis / Q000276:immunology; D053146:Myeloblastin / Q000276:immunology; D009195:Peroxidase / Q000276:immunology
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D000911:Antibodies, Monoclonal; D001324:Autoantigens; D003182:Complement C5; D004268:DNA-Binding Proteins; C499308:Rag2 protein, mouse; D015936:Complement C5a; D003165:Complement System Proteins; D009195:Peroxidase; D053146:Myeloblastin