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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Prof. Dr. Peter R. Galle
I. Medizinische Klinik und Poliklinik, Johannes Gutenberg-Universität
Langenbeckstraße 1
55101 Mainz
Phone: ++ 49/61 31/17 72 75/6
Fax: ++ 49/61 31/17 55 95
Email: galle@uni-mainz.de
