PMID- 23138554 OWN - NLM STAT- MEDLINE DCOM- 20130509 LR - 20211021 IS - 1435-1250 (Electronic) IS - 0340-1855 (Linking) VI - 71 IP - 9 DP - 2012 Nov TI - [Update Churg-Strauss syndrome]. PG - 765-70 LID - 10.1007/s00393-012-0985-9 [doi] AB - 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. FAU - Moosig, F AU - Moosig F AD - Klinik fur Rheumatologie und Immunologie, Klinikum Bad Bramstedt GmbH, Universitatsklinikum Schleswig Holstein, Oskar-Alexander-Str. 26, 24576, Bad Bramstedt, Deutschland. F.Moosig@klinikumbb.de FAU - Hellmich, B AU - Hellmich B LA - ger PT - English Abstract PT - Journal Article TT - Update Churg-Strauss-Syndrom. PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 RN - 0 (Immunosuppressive Agents) SB - IM MH - Churg-Strauss Syndrome/complications/*diagnosis/*drug therapy MH - Eosinophilia/*diagnosis/etiology/*prevention & control MH - Humans MH - Immunosuppressive Agents/*therapeutic use MH - Multiple Organ Failure/*diagnosis/etiology/*prevention & control EDAT- 2012/11/10 06:00 MHDA- 2013/05/10 06:00 CRDT- 2012/11/10 06:00 PHST- 2012/11/10 06:00 [entrez] PHST- 2012/11/10 06:00 [pubmed] PHST- 2013/05/10 06:00 [medline] AID - 10.1007/s00393-012-0985-9 [doi] PST - ppublish SO - Z Rheumatol. 2012 Nov;71(9):765-70. doi: 10.1007/s00393-012-0985-9.