Autoimmune Lebererkrankungen – ein Update (Teil 2)

Im 1. Teil der Reihe „Autoimmune Lebererkrankungen – ein Update (Teil 1)“ wurden die Grundlagen zur Diagnostik und Surveillance autoimmuner Lebererkrankungen und die autoimmune Hepatitis (AIH) behandelt [1]. In diesem 2. Teil werden mit der primär biliären Cholangitis (PBC) und der primär sklerosierenden Cholangitis (PSC) cholestatische autoimmune Lebererkrankungen beleuchtet.

Kernaussagen

• Zur Therapie der primär biliären Cholangitis (PBC) stehen Ursodesoxycholsäure (UDCA) mit einem guten Wirkungs-/
  Nebenwirkungs-Profil, Obeticholsäure (OCA) sowie Bezafibrat (Off-Label-Use) zur Verfügung.

• Besondere Beachtung erfordern PBC-assoziierte Symptome wie Sicca-Symptomatik, Fatigue und Juckreiz. Letzterer lässt sich durch die Gabe von Bezafibrat adressieren.

• Eine effektive medikamentöse Therapie der primär sklerosierenden Cholangitis (PSC) steht nicht zur Verfügung. Das Management konzentriert sich daher

  • erstens auf das Erkennen, Abklären und Behandeln hochgradiger und klinisch relevanter Gallengangstrikturen,

  • zweitens auf die Durchführung von Vorsorgeuntersuchungen zur frühzeitigen Detektion hepatobiliärer und kolorektaler Tumoren.

• Bei Vorliegen einer relevanten Stenose (bildmorphologische Einengung auf mehr als 75% und Zeichen der Cholestase/Cholangitis) sollte bei der primär sklerosierenden Cholangitis eine endoskopische retrograde Cholangiopankreatikografie (ERCP) erwogen werden. Die Ballondilatation ist der Stenteinlage überlegen, weshalb auf Letztere, wenn möglich, verzichtet werden sollte. Falls eine Stenteinlage notwendig ist, sollte diese zeitlich limitiert bleiben.

• Insbesondere bei Patient*innen mit primär sklerosierender Cholangitis (PSC) sowie Patient*innen mit autoimmuner Hepatitis (AIH) und primär biliärer Cholangitis (PBC) mit unzureichendem Therapieansprechen sollte eine Anbindung an ein erfahrenes Zentrum erfolgen und der Einschluss in klinische Studien geprüft werden.

• Der MELD-Score (Model of End Stage Liver Disease) bildet den Schweregrad der primär sklerosierenden Cholangitis oft nur unzureichend ab. Eine Listung zur Lebertransplantation ist deshalb zusätzlich über Standard-Exception-Kriterien möglich (≥ 2 Cholangioseptitiden, Entwicklung einer dominanten Stenose, Reduktion ≥ 10% des Body-Mass-Indexes in 12 Monaten; 2 von 3 Kriterien müssen erfüllt sein).

Schlüsselwörter

primär biliäre Cholangitis - primär sklerosierende Cholangitis - hepatischer Pruritus

Publication History

Article published online:
11 October 2022

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