title: [Update Churg-Strauss syndrome].
abstract: The Churg-Strauss syndrome (CSS) is the rarest subtype of the so-called anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) and has the lowest frequency of ANCA-positivity (around 30%). In addition to asthma and blood eosinophilia, CSS is characterized by end-organ damage, which can be caused by either vasculitis and/or tissue infiltration of eosinophilic granulocytes. The CSS shares many etiological and clinical features of other hypereosinophilic syndromes. Recently, a distinct genetic background could be demonstrated for both the ANCA-positive and ANCA-negative subtypes of CSS as compared to the other two forms of AAV. Among other cytokines, interleukin-5 (IL-5) could be identified as a key mediator of eosinophilia. Therefore, recent clinical trials in CSS aimed to target IL-5. Outside of clinical trials, treatment of CSS is adapted to disease stage and activity, as recommended for other types of AAV.
authors.affiliation: Klinik für Rheumatologie und Immunologie, Klinikum Bad Bramstedt GmbH, Universitätsklinikum Schleswig Holstein, Oskar-Alexander-Str. 26, 24576, Bad Bramstedt, Deutschland. F.Moosig@klinikumbb.de
authors.forename: F
authors.initials: F
authors.lastname: Moosig
authors.forename: B
authors.initials: B
authors.lastname: Hellmich
mesh_terms: D015267:Churg-Strauss Syndrome / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D004802:Eosinophilia / Q000175:diagnosis* / Q000209:etiology / Q000517:prevention & control*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D009102:Multiple Organ Failure / Q000175:diagnosis* / Q000209:etiology / Q000517:prevention & control*
chemical_list: D007166:Immunosuppressive Agents