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Die oropharyngeale oder \u00f6sophageale Dysphagie bei IIM ist mit Aspiration und Malnutrition assoziiert. Sie spricht mit Ausnahme der Dysphagie bei \u201einclusion body myositis\u201c gut auf konventionelle Immunmodulation an. Die seltene intestinale Vaskulopathie bei NXP2-assoziierter Dermatomyositis bei Kindern und Erwachsenen f\u00fchrt zu lebensbedrohlichen Perforationen und Blutungen vom \u00d6sophagus bis zum Dickdarm und ist daher mit einer hohen Mortalit\u00e4t verbunden. Sie spricht nicht gut auf konventionelle Immunmodulation an. In den letzten Jahren zeigten experimentelle Therapieans\u00e4tze mit Januskinase(JAK)-Inhibitoren (durch Interferon-Hemmung), Vedolizumab und dem C5-Inhibitor Eculizumab jedoch vielversprechende Resultate. Die ebenfalls seltene Pneumatosis cystoides intestinalis zeigt sich durch gasgef\u00fcllte Zysten in der Submukosa v.\u202fa. von D\u00fcnn- und Dickdarm und ist bei IIM h\u00e4ufig aber nicht immer ein asymptomatischer Zufallsbefund, der konservativen Ma\u00dfnahmen zug\u00e4nglich ist. IIM k\u00f6nnen mit Dys- und Hypomotilit\u00e4t im gesamten Gastrointestinaltrakt verbunden sein, wobei am Darm Pseudoobstruktion und bakterielle \u00dcberwucherung mit Malnutrition entstehen k\u00f6nnen. Wichtig zu beachten ist, dass bei IIM mit erh\u00f6hter Kreatinkinase auch Aspartat-Aminotransferase (ASAT) und Alanin-Aminotransferase (ALAT) aus dem Skelettmuskel freigesetzt werden und eine Transaminasen-Erh\u00f6hung daher praktisch nie Ausdruck einer Hepatopathie ist.<\/jats:p>","DOI":"10.1007\/s00393-026-01884-4","type":"journal-article","created":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T15:14:30Z","timestamp":1792682070000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Gastrointestinale Manifestationen bei idiopathisch-inflammatorischen Myopathien","Gastrointestinal manifestations in idiopathic inflammatory myopathies"],"prefix":"10.1007","author":[{"given":"Pascal","family":"Seitz","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,10,22]]},"reference":[{"issue":"1","key":"1884_CR1","doi-asserted-by":"publisher","first-page":"113","DOI":"10.1016\/j.rdc.2017.09.006","volume":"44","author":"C Gadiparthi","year":"2018","unstructured":"Gadiparthi\u00a0C, Hans\u00a0A, Potts\u00a0K et\u00a0al (2018) Gastrointestinal and Hepatic Disease in the Inflammatory Myopathies. 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