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Gastroenterologie up2date 2022; 18(03): 225-241
DOI: 10.1055/a-1364-8489
Leber/Galle/Pankreas

Autoimmune Lebererkrankungen – ein Update (Teil 1)

Autoimmune Hepatitis (AIH) und allgemeines Vorgehen bei immunvermittelten Lebererkrankungen
Silja Steinmann‡
,
Ida Schregel‡
,
Christoph Schramm
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Zu den autoimmunen Lebererkrankungen gehören die autoimmune Hepatitis (AIH), die primär biliäre Cholangitis (PBC) und die primär sklerosierende Cholangitis (PSC). Diagnostik, Therapie und Surveillance dieser Erkrankungen sind aufwendig. Dieser Beitrag widmet sich der Versorgung der autoimmunen Lebererkrankungen mit dem Fokus auf neue klinische Aspekte über die kondensierte Darstellung der aktuellen leitliniengerechten Versorgung hinaus.
Kernaussagen

  • Autoimmune Lebererkrankungen zeigen eine zunehmende Prävalenz und erfordern in der Versorgung eine interdisziplinäre Zusammenarbeit. Das Europäische Referenznetzwerk für seltene Lebererkrankungen (ERN RARE-LIVER) unterstützt und fördert dies im europäischen Raum.

  • Zu den autoimmunen Lebererkrankungen gehören die autoimmune Hepatitis (AIH), die primär biliäre Cholangitis (PBC) und die primär sklerosierende Cholangitis (PSC).

  • Die AIH kann sich klinisch wie auch histologisch heterogen präsentieren. Eine immunsuppressive Therapie ist der wichtigste Schritt, um die Entstehung einer Leberzirrhose zu verhindern und die leberassoziierte Mortalität zu senken. Mittels transienter Elastografie kann der Krankheitsprogress im Langzeitverlauf erfasst werden.

  • Das Therapieansprechen der AIH sollte regelmäßig laborchemisch überprüft werden. Eine Senkung der Transaminasen um weniger als 80% des Ausgangswertes nach einer Therapiedauer von 8 Wochen ist mit einem schlechteren Outcome assoziiert. Ein komplettes laborchemisches Therapieansprechen (= normale Transaminasen und normales IgG) sollte nach 6 Monaten, spätestens nach 12 Monaten erreicht werden.

  • Azathioprin ist das Medikament der 1. Wahl zur Remissionserhaltung der AIH. Die Bestimmung der Azathioprinmetaboliten kann die Therapiesteuerung unterstützen.

  • Bei Patient*innen mit PSC sowie bei Vorliegen einer Zirrhose bei AIH und PBC kommt der regelmäßigen Tumorsurveillance eine wichtige Rolle zu.

Schlüsselwörter

Immundiagnostik - autoimmune Lebererkrankung - autoimmune Hepatitis - unklare Hepatopathie


Publication History

Article published online:
11 October 2022

© 2022. Thieme. All rights reserved.

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