Die primär sklerosierende Cholangitis als Präkanzerose

Zusammenfassung

Die primär sklerosierende Cholangitis (PSC) ist eine chronische, cholestatische Lebererkrankung, deren Pathogenese unklar ist. Sie verläuft in der Regel progredient und führt im Mittel innerhalb von 12 Jahren zum Tod oder zur Lebertransplantation. Die PSC geht zudem mit einer deutlich erhöhten Inzidenz hepatobiliärer und, bei Vorliegen einer Colitis ulcerosa, auch kolorektaler Karzinome einher. Sie kann daher als Präkanzerose angesehen werden. In der folgenden Übersicht soll insbesondere auf das Risiko für die Entwicklung eines Cholangiokarzinoms und die damit verbundenen diagnostischen und therapeutischen Herausforderungen bei Patienten mit PSC eingegangen werden.

Abstract

Primary sclerosing cholangitis is a chronic cholestatic liver disease of unknown aetiology. The course of the disease is usually progressive with the development of liver cirrhosis leading to death or liver transplantation within an average of 12 years. To date it is well known that the development of hepatobiliary malignancies and the rate of colonic mucosal dysplasia and carcinoma in patients with concomitant ulcerative colitis are greatly enhanced in patients with PSC. PSC can therefore be regarded as a premalignant condition. The following review will focus on the development of cholangiocellular carcinoma in patients with PSC and the associated diagnostic and therapeutic challenges.

Schlüsselwörter

primär sklerosierende Cholangitis - Cholangiokarzinom - Prävalenz - Tumormarker - CEA - CA19-9 - Bürstenzytologie - Lebertransplantation - Ursodesoxycholsäure - UDCA

Key words

Primary sclerosing cholangitis - cholangiocarcinoma - prevalence - tumor markers - CEA - CA19 - 9 - brush cytology - liver transplantation - ursodeoxycholic acid - UDCA

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