Cholestatische Lebererkrankungen

Zusammenfassung

Die primär biliäre Cholangitis (PBC) und die primär sklerosierende Cholangitis (PSC) werden als cholestatische Lebererkrankungen bezeichnet und den autoimmunen Lebererkrankungen zugerechnet. Die Pathogenese ist bislang nur unvollständig charakterisiert. Patienten sind klinisch oft asymptomatisch oder zeigen die Leitsymptome Juckreiz und Müdigkeit. Die Diagnose der PBC beruht auf dem Nachweis einer erhöhten alkalischen Phosphatase und antimitochondrialer Antikörper (AMA). Standardtherapie ist Ursodeoxycholsäure. Die Diagnose der PSC wird bei erhöhter alkalischer Phosphatase durch ein typisches Cholangiogramm gesichert. Eine Leberbiopsie ist jeweils nur bei unklaren Befunden indiziert. Die PSC ist in 80 % mit einer chronisch entzündlichen Darmerkrankung assoziiert. Eine medikamentöse Therapie ist nicht empfohlen, wenngleich Ursodeoxycholsäure häufig eingesetzt wird. Die endoskopische Therapie von Gallengangsstrikturen steht im Vordergrund. Die gemeinsame Endstrecke ist die Leberzirrhose, wobei die Lebertransplantation frühzeitig erwogen werden sollte.

Abstract

Common cholestatic liver diseases are primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). Both diseases are considered autoimmune disorders; however, the precise pathogenesis remains elusive. Patients usually show no symptoms or present with pruritus and fatigue. High alkaline phosphatase and the presence of antimitochondrial antibodies are sufficient to diagnose PBC. Ursodeoxycholic acid is approved and recommended for the treatment of PBC. The diagnosis of PSC is established by elevated alkaline phosphatase-levels and typical cholangiographic findings. Liver biopsy is not generally necessary for the diagnosis. The prevalence of inflammatory bowel disease in PSC is up to 80 %. There is no established pharmacological therapy, although Ursodeoxycholic acid is often prescribed. Endoscopic management of biliary obstructions offers clinical benefit. Liver transplantation is the definitive treatment for patients with advanced or decompensated cirrhosis.

Schlüsselwörter

primär biliäre Cholangitis - primär sklerosierende Cholangitis - Cholestase - Ursodeoxycholsäure

Keywords

primary biliary cholangitis - primary sclerosing cholangitis - cholestasis - ursodeoxycholic acid

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