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Erschienen in: Der Pneumologe 5/2018

20.06.2018 | Myokarditis | Leitthema

Eosinophile Granulomatose und Polyangiitis – Diagnostik und Therapie

verfasst von: Prof. Dr. Dr. C. Kroegel, H. Slevogt, M. Foerster, R. Ali, T. Neumann, U. Costabel

Erschienen in: Zeitschrift für Pneumologie | Ausgabe 5/2018

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Zusammenfassung

Bei der eosinophilen Granulomatose mit Polyangiitis (EGPA; früher Churg-Strauss-Syndrom) handelt es sich um eine systemische Angiitis der kleinen Gefäße mit extravaskulären Granulomen und Hypereosinophilie. Klinisch stehen eine chronische Rhinosinusitis und ein progressiv fortschreitendes, kortisonabhängiges Asthma im Vordergrund. Zusätzlich können sich im Verlauf eine palpale Purpura (kutane Vaskulitis), eine Mononeuritis multiplex, eine Myokarditis und/oder eine Glomerulonephritis entwickeln. Laborchemisch zeigt sich eine zunehmende Blut- und Gewebeeosinophilie. Antineutrophile zytoplasmatische Antikörper (p-ANCA) sind in 30–40 % der Fälle nachweisbar. Die Diagnose beruht auf den Klassifikationskriterien des American College of Rheumatology (ACR) in Verbindung mit dem histologischen oder klinischen Nachweis einer Vaskulitis. Die Therapie beruht auf einer immunsuppressiven Standardtherapie (Kortison/Immunsuppressiva) und einer Immunmodulation mittels Biologika.
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Metadaten
Titel
Eosinophile Granulomatose und Polyangiitis – Diagnostik und Therapie
verfasst von
Prof. Dr. Dr. C. Kroegel
H. Slevogt
M. Foerster
R. Ali
T. Neumann
U. Costabel
Publikationsdatum
20.06.2018

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