Send to

Choose Destination
Z Rheumatol. 2012 Nov;71(9):765-70. doi: 10.1007/s00393-012-0985-9.

[Update Churg-Strauss syndrome].

[Article in German]

Author information

Klinik f├╝r Rheumatologie und Immunologie, Klinikum Bad Bramstedt GmbH, Universit├Ątsklinikum Schleswig Holstein, Oskar-Alexander-Str. 26, 24576, Bad Bramstedt, Deutschland.


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.

[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Springer
Loading ...
Support Center