title: Drugs that inhibit complement.
abstract: The complement system is an important part of the innate immune system. Complement plays a crucial role in the pathophysiology of many disorders. Despite the pivotal role of the complement system, an approved targeted inhibitor of a complement factor became available only recently. Eculizumab is a humanized monoclonal antibody that inhibits complement factor C5. It is a targeted, disease modifying, treatment of paroxysmal nocturnal hemoglobinuria (PNH). It was approved be the US FDA and the European Commission in 2007. In this review we will update the experience with eculizumab in PNH and discuss potential use of eculizumab in other disorders (e.g. cold agglutinin disease; atypical HUS) and new approaches to complement inhibition with drugs other than eculizumab.
authors.affiliation: German Red Cross Blood Transfusion Service Baden-Württemberg-Hessia, Institute of Clinical Transfusion Medicine and Immunogenetics Ulm, and University of Ulm, Helmholtzstraße 10, 89081 Ulm, Germany. h.schrezenmeier@blutspende.de
authors.forename: Hubert
authors.initials: H
authors.lastname: Schrezenmeier
authors.forename: Britta
authors.initials: B
authors.lastname: Höchsmann
mesh_terms: D000744:Anemia, Hemolytic, Autoimmune / Q000188:drug therapy / Q000378:metabolism; D000818:Animals; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D003182:Complement C5 / Q000037:antagonists & inhibitors* / Q000378:metabolism; D051056:Complement Inactivating Agents / Q000627:therapeutic use*; D006457:Hemoglobinuria, Paroxysmal / Q000188:drug therapy* / Q000378:metabolism; D006463:Hemolytic-Uremic Syndrome / Q000188:drug therapy / Q000378:metabolism; D006801:Humans
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D003182:Complement C5; D051056:Complement Inactivating Agents; C481642:eculizumab