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Die PSC ist charakterisiert durch genetische Risikofaktoren, Ver\u00e4nderungen des Mikrobioms und eine pathologische Immunaktivierung, die zur Entwicklung einer bili\u00e4ren Fibrose f\u00fchren. Cholangiozyten spielen dabei eine aktive Rolle f\u00fcr die Inflammationsprozesse, da sie direkt mit dem Mikrobiom und Immunzellen in Interaktion stehen. Die begleitende chronisch-entz\u00fcndliche Darmerkrankung weist einen distinkten Ph\u00e4notyp auf und bewirkt \u00fcber Ver\u00e4nderungen der Darmbarriere und des enterohepatischen Kreislaufs der Gallens\u00e4uren eine wechselseitige Modulation intestinaler und hepatischer Krankheitsaktivit\u00e4t. W\u00e4hrend die aktuell zur Verf\u00fcgung stehenden medikament\u00f6sen und endoskopischen Therapieoptionen nur symptomatisch wirksam sind, befinden sich neue pharmakologische Therapien in klinischer Evaluation. In dem vorliegenden Review wollen wir einen Einblick in das aktuelle Verst\u00e4ndnis der Pathogenese der PSC sowie neue therapeutische Entwicklungen geben.<\/jats:p>","DOI":"10.1055\/a-2730-4719","type":"journal-article","created":{"date-parts":[[2026,1,26]],"date-time":"2026-01-26T23:32:11Z","timestamp":1769470331000},"page":"56-66","source":"Crossref","is-referenced-by-count":1,"title":["Immunpathogenese und Therapie der Prim\u00e4r Sklerosierenden Cholangitis"],"prefix":"10.1055","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7248-7266","authenticated-orcid":false,"given":"Britta F.","family":"Zecher","sequence":"additional","affiliation":[{"name":"I. Medizinische Klinik und Poliklinik, Universit\u00e4tsklinikum Hamburg-Eppendorf, Hamburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Schramm","sequence":"additional","affiliation":[{"name":"I. Medizinische Klinik und Poliklinik, Universit\u00e4tsklinikum Hamburg-Eppendorf, Hamburg, Germany"},{"name":"Hamburg Center for Translational Immunology, Universit\u00e4tsklinikum Hamburg-Eppendorf, Hamburg, Germany"},{"name":"Martin Zeitz Centrum f\u00fcr Seltene Erkrankungen, Universit\u00e4tsklinikum Hamburg-Eppendorf, Hamburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2026,1,26]]},"reference":[{"key":"ref2","doi-asserted-by":"publisher","first-page":"1956","DOI":"10.3748\/wjg.v21.i6.1956","article-title":"Distinctive inflammatory bowel disease phenotype in primary sclerosing cholangitis","volume":"21","author":"AB de Vries","year":"2015","journal-title":"World J Gastroenterol"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"2045","DOI":"10.1002\/hep.26565","article-title":"Population-based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis","volume":"58","author":"K 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