title: [Update: Behçet's disease].
abstract: Behçet's disease (BD) is now mentioned in the latest Chapel Hill nomenclature of vasculitides and is classified under the variable vessel vasculitides (VVV). Pathogenetically, a new classification among the so-called mixed-pattern diseases between classical polygenic autoinflammatory disorders and autoimmune diseases is being discussed. The genetic association with HLA-B51 is undisputed and an association with HLA-A26 as well as with polymorphisms in the IL-10 and IL23R-IL12RB2 genes have recently been described. Increasingly, a participation of IL-17 in the pathogenesis of BD is assumed. Therapeutically, the EULAR recommendations are still applicable. Interferon-alpha can be discontinued for severe ocular BD in remission without further relapses. Infliximab can be switched to adalimumab effectively and recent case series show an efficacy of IL-1 antagonists, tocilizumab and rituximab for BD.
authors.affiliation: Zentrum für Interdisziplinäre Rheumatologie Stuttgart (ZIRS), Rheumatologische Schwerpunktpraxis, Seelbergstr. 11, 70372, Stuttgart, Deutschland. ina.koetter@uni-tuebingen.de
authors.forename: I
authors.initials: I
authors.lastname: Kötter
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D003937:Diagnosis, Differential; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D013167:Spondylitis, Ankylosing / Q000145:classification / Q000175:diagnosis* / Q000188:drug therapy*; D009626:Terminology as Topic
chemical_list: D000911:Antibodies, Monoclonal; D007166:Immunosuppressive Agents