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Eosinophile Granulomatose und Polyangiitis – Diagnostik und Therapie

Eosinophilic granulomatosis with polyangiitis—diagnosis and therapy

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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.

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA, Churg–Strauss Syndrome) is an allergic necrotizing granulomatous angiitis of the small vessels associated with eosinophilia. EGPA initially presents with upper airway tract and lung involvement, which often progresses over many years and eventually requires systemic glucocorticoid treatment. In addition to the respiratory tract, any organ system can be affected, leading to vasculitic skin lesions, peripheral neuropathy (mononeuritis multiplex), eosinophilic myocarditis, and glomerulonephritis. Progressive blood and tissue eosinophilia is found in almost all cases, while perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) may be present in 30–40% of the patients. The diagnosis is based on the criteria proposed by the American College of Rheumatology (ACR). In addition, application of these criteria requires the histological evidence of vasculitis or surrogate features of vasculitis. Long-term treatment is based either on immunosuppressive standard therapy (glucocorticoids/immunosuppressants) or immunomodulation using biologicals.

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Authors and Affiliations

  1. Abt. Pneumologie & Allergologie/Immunologie, Medizinische Klinik I, Universitätsklinkum Jena, Am Klinikum 1, 07740, Jena, Deutschland

    C. Kroegel, M. Foerster & R. Ali

  2. AG Host Septomics, Universitätsklinikum Jena, Jena, Deutschland

    H. Slevogt

  3. Abt. für Rheumatologie, Immunologie und Rehabilitation, Kantonsspital St. Gallen, St. Gallen, Schweiz

    T. Neumann

  4. Ruhrlandklinik, Westdeutsches Lungenzentrum am Universitätsklinikum Essen, Essen, Deutschland

    U. Costabel

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  1. C. Kroegel
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  2. H. Slevogt
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  3. M. Foerster
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  4. R. Ali
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  5. T. Neumann
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Correspondence to C. Kroegel.

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Interessenkonflikt

C. Kroegel, H. Slevogt, M. Foerster, R. Ali, T. Neumann und U. Costabel geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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J. Schreiber, Magdeburg

C. Kroegel, Jena

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Kroegel, C., Slevogt, H., Foerster, M. et al. Eosinophile Granulomatose und Polyangiitis – Diagnostik und Therapie. Pneumologe 15, 333–343 (2018). https://doi.org/10.1007/s10405-018-0194-6

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