Immunpathogenese und Therapie der Primär Sklerosierenden Cholangitis

Zusammenfassung

Die Primär Sklerosierende Cholangitis (PSC) ist eine immunvermittelte Erkrankung der Gallenwege, für die bisher keine Prognose-verbessernden Therapien zur Verfügung stehen. Die PSC ist charakterisiert durch genetische Risikofaktoren, Veränderungen des Mikrobioms und eine pathologische Immunaktivierung, die zur Entwicklung einer biliären Fibrose führen. Cholangiozyten spielen dabei eine aktive Rolle für die Inflammationsprozesse, da sie direkt mit dem Mikrobiom und Immunzellen in Interaktion stehen. Die begleitende chronisch-entzündliche Darmerkrankung weist einen distinkten Phänotyp auf und bewirkt über Veränderungen der Darmbarriere und des enterohepatischen Kreislaufs der Gallensäuren eine wechselseitige Modulation intestinaler und hepatischer Krankheitsaktivität. Während die aktuell zur Verfügung stehenden medikamentösen 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 Verständnis der Pathogenese der PSC sowie neue therapeutische Entwicklungen geben.

Abstract

Primary sclerosing cholangitis (PSC) is an immune-mediated disease of the biliary tract for which no prognosis-improving therapies are yet available. PSC is characterized by genetic risk factors, alterations in the microbiome and pathological immune activation leading to the development of biliary fibrosis. Cholangiocytes play an active role in the inflammatory processes, as they interact directly with the microbiome and immune cells. The concomitant chronic inflammatory bowel disease has a distinct phenotype and causes a reciprocal modulation of intestinal and hepatic disease activity via changes in the intestinal barrier and the enterohepatic circulation of bile acids. While the currently available drugs and endoscopic treatment options are only symptomatically effective, new pharmacologic therapies are under clinical evaluation. In this review, we aim to provide an insight into the current understanding of the pathogenesis of PSC and new therapeutic developments.

Schlüsselwörter

Leber - CED - Darm

Keywords

Liver - IBD - Intestine

Publication History

Received: 07 August 2025

Accepted after revision: 23 October 2025

Article published online:
26 January 2026

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