{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,6,26]],"date-time":"2025-06-26T22:24:58Z","timestamp":1750976698518,"version":"3.28.0"},"reference-count":50,"publisher":"Georg Thieme Verlag KG","issue":"10","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Laryngorhinootologie"],"published-print":{"date-parts":[[2024,10]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title><jats:p>Die eosinophile Granulomatose mit Polyangiitis (EGPA) ist eine seltene Form der ANCA-assoziierten Vaskulitiden (AAV) aus der Gruppe der Kleingef\u00e4\u00dfvaskulitiden. Sie ist definiert als eine Vaskulitis kleiner und mittelgro\u00dfer Gef\u00e4\u00dfe mit granulomat\u00f6ser Entz\u00fcndung sowie Blut- und Gewebseosinophilie. Fast alle Patienten haben allergische Symptome mit Asthma bronchiale und rhinosinusitischen Beschwerden. Das sonstige klinische Spektrum variiert je nach Lokalisation, Schweregrad und Art der Krankheitsmanifestation. Durch die eosinophile Infiltration und Inflammation kann es zu Rhinosinusitis, Pneumonitis, gastrointestinaler Beteiligung und Kardiomyopathie kommen. Insbesondere Letztere ist mit einer schlechteren Prognose assoziiert. Als nekrotisierende pauci-immune Kleingef\u00e4\u00dfvaskulitis kann die EGPA, \u00e4hnlich wie die anderen AAV, pulmonale Infiltrate mit alveol\u00e4rer H\u00e4morrhagie, eine Glomerulonephritis, kutane Vaskulitis mit Purpura, sowie zentrale und periphere neurologische Sch\u00e4digungen verursachen. Das Auftreten perinukle\u00e4rer ANCA (pANCA), mit Spezifit\u00e4t gegen die Myeloperoxidase (MPO) wird in etwa einem Drittel der F\u00e4lle beobachtet, ist allerdings nicht spezifisch f\u00fcr die EGPA. MPO-ANCA-positive Patienten haben h\u00e4ufiger eine periphere neurologische Beteiligung und eine Glomerulonephritis, w\u00e4hrend ANCA-negative Patienten h\u00e4ufiger eine kardiale und pulmonale Beteiligung aufweisen. Eine differentialdiagnostische Herausforderung im klinischen Alltag stellt die Abgrenzung zum hypereosinophilen Syndrom (HES) dar. Das Therapiekonzept der EGPA h\u00e4ngt davon ab, ob der Schweregrad der Erkrankung potentiell Organ- oder lebensbedrohlich ist. Bei schweren Formen der EGPA werden in der Akuttherapie haupts\u00e4chlich Glukokortikoide in Kombination mit Cyclophosphamid eingesetzt. Rituximab wird mittlerweile als alternative Behandlungsoption in den Leitlinien genannt. Zur Remissionserhaltung stehen verschiedene immunsuppressive Therapien zur Verf\u00fcgung. Bei EGPA ohne schwere Organbeteiligung ist die IL-5 Blockade mit Mepolizumab zugelassen.<\/jats:p>","DOI":"10.1055\/a-2214-1669","type":"journal-article","created":{"date-parts":[[2024,7,4]],"date-time":"2024-07-04T22:53:55Z","timestamp":1720133635000},"page":"705-714","source":"Crossref","is-referenced-by-count":1,"title":["Eosinophile Granulomatose mit Polyangiitis \u2013 ein \u00dcbersichtsartikel"],"prefix":"10.1055","volume":"103","author":[{"given":"Viktoria","family":"Schindler","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Rheumatologie und Klinische Immunologie, Department Innere Medizin, Universit\u00e4tsklinikum Freiburg, Freiburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nils","family":"Venhoff","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Rheumatologie und Klinische Immunologie, Department Innere Medizin, Universit\u00e4tsklinikum Freiburg, Freiburg, 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