title: [Complement deficiencies and human diseases].
abstract: The complement system is a complex system involving serum and membrane proteins interacting in a regulated manner. The complement system plays a major role in antibacterial immunity, in inflammation, and in immune complex processing. Therefore, deficiencies in complement proteins are associated with increased susceptibility to bacterial infections and autoimmune diseases. These deficiencies can be inherited or acquired. Most of them can be screened by simple laboratory tests but require a diagnosis in a specialized laboratory. All sequences of complement genes are known, and the discovery of a deficiency must lead to genetic testing. The discovery of a congenital deficiency requires a familial study and a prophylaxis. In this article, we review the complement cascade, the laboratory tests to explore it, and the main diseases associated with complement deficiencies.
authors.affiliation: Immunologie biologique, Hôpital européen Georges-Pompidou, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors.forename: Jérémie
authors.initials: J
authors.lastname: Rosain
authors.affiliation: Immunologie biologique, Hôpital européen Georges-Pompidou, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Ngo
authors.affiliation: Immunologie biologique, Hôpital européen Georges-Pompidou, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors.forename: Pauline
authors.initials: P
authors.lastname: Bordereau
authors.affiliation: Immunologie biologique, Hôpital européen Georges-Pompidou, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors.forename: Nelly
authors.initials: N
authors.lastname: Poulain
authors.affiliation: Immunologie biologique, Hôpital européen Georges-Pompidou, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors.forename: Stéphane
authors.initials: S
authors.lastname: Roncelin
authors.affiliation: Immunologie biologique, Hôpital européen Georges-Pompidou, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors.forename: Paula
authors.initials: P
authors.lastname: Vieira Martins
authors.affiliation: Immunologie biologique, Hôpital européen Georges-Pompidou, Assistance Publique-Hôpitaux de Paris, Paris, France, Inserm UMR_S1138, Centre de recherche des Cordeliers, Paris, France.
authors.forename: Marie-Agnès
authors.initials: MA
authors.lastname: Dragon-Durey
authors.affiliation: Immunologie biologique, Hôpital européen Georges-Pompidou, Assistance Publique-Hôpitaux de Paris, Paris, France, Inserm UMR_S1138, Centre de recherche des Cordeliers, Paris, France.
authors.forename: Véronique
authors.initials: V
authors.lastname: Frémeaux-Bacchi
mesh_terms: D001327:Autoimmune Diseases / Q000276:immunology; D001424:Bacterial Infections / Q000276:immunology; D003167:Complement Activation / Q000276:immunology; D003165:Complement System Proteins / Q000172:deficiency* / Q000276:immunology; D004198:Disease Susceptibility / Q000276:immunology; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000276:immunology
chemical_list: D003165:Complement System Proteins
keywords: angioedema; autoimmune diseases; complement; immune deficiencies; renal diseases