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Erschienen in: Zeitschrift für Rheumatologie 9/2012

01.11.2012 | Leitthema

Update Churg-Strauss-Syndrom

verfasst von: PD Dr. F. Moosig, B. Hellmich

Erschienen in: Zeitschrift für Rheumatologie | Ausgabe 9/2012

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Zusammenfassung

Das Churg-Strauss-Syndrom ist die seltenste der sog. ANCA-assoziierten Vaskulitiden (AAV) und weist die niedrigste Frequenz an ANCA-Positivität (ca. 30%) auf. Neben Asthma bronchiale und Bluthypereosinophilie ist es durch Endorganschäden gekennzeichnet, die sowohl vaskulitischer Genese als auch durch Gewebeinfiltration durch eosinophile Granulozyten verursacht sein können. Klinisch und ätiologisch besteht eine Nähe zu den hypereosinophilen Syndromen. In jüngerer Zeit konnte ein gegenüber den anderen AAV distinkter genetischer Hintergrund nachgewiesen werden, der auch bei ANCA-positiven und ANCA-negativen Formen unterschiedlich ist. Als ein wesentlicher Mediator der Hypereosinophilie konnte neben anderen Zytokinen das IL-5 identifiziert werden. Daher zielen aktuelle Therapiestudien auf eine Neutralisation von IL-5. Außerhalb von Studien erfolgt die Behandlung stadien- und aktivitätsadaptiert, wie auch für die übrigen AAV empfohlen.
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Metadaten
Titel
Update Churg-Strauss-Syndrom
verfasst von
PD Dr. F. Moosig
B. Hellmich
Publikationsdatum
01.11.2012
Verlag
Springer-Verlag
Erschienen in
Zeitschrift für Rheumatologie / Ausgabe 9/2012
Print ISSN: 0340-1855
Elektronische ISSN: 1435-1250
DOI
https://doi.org/10.1007/s00393-012-0985-9

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