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Primär sklerosierende Cholangitis

  • Schwerpunkt: Gallenwegserkrankungen
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Zusammenfassung

Die primär sklerosierende Cholangitis (PSC) ist eine chronische cholestatische Lebererkrankung, die durch fibrosierende Entzündung und Obliteration der intra- und/oder extrahepatischen Gallengänge charakterisiert ist. Die Erkrankung gehört zu den häufigsten cholestatischen Erkrankungen im Erwachsenenalter und wird gegenwärtig mit zunehmender Häufigkeit diagnostiziert. Sie ist oft mit einer Colitis ulcerosa assoziiert. Patienten mit PSC haben eine erhöhte Inzidenz von Gallengangskarzinomen, jene mit Colitis ulcerosa eine erhöhte Inzidenz von Kolonkarzinomen. Die immunosuppressive Behandlung ist wenig effektiv. Ursodeoxycholsäure (UDC) verbessert die Leberhistologie bei der PSC. Patienten sollten so früh wie möglich behandelt werden, um ein Progression in die fortgeschrittenen Stadien der Erkrankung zu verhindern. Stenosen großer Gallengänge können erfolgreich endoskopisch behandelt werden. Bei Patienten im Endstadium der Erkrankung ist UDC nicht wirksam und eine Lebertransplantation ist indiziert.

Abstract

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease, characterized by fibrosing inflammation and obliteration of intra and/or extrahepatic bile ducts. The disease belongs to the most common cholestatic diseases in adults and at present is diagnosed with increasing frequency. It is very often associated with ulcerative colitis. Patients with PSC have an increased incidence of bile duct carcinomas and those with ulcerative colitis also have an increased incidence of colonic carcinomas. Immunosuppressive treatment is little effective. Ursodeoxycholic acid (UDCA) has been shown to improve liver histology in PSC. The aim is to treat patients as early as possible to prevent progression to the advanced stages of the disease. During treatment with UDCA stenoses of major ducts may develop and early endoscopic dilatation is highly effective. In patients with endstage disease, UDCA is not effective and liver transplantation is indicated.

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Authors and Affiliations

  1. Medizinische Universitätsklinik Heidelberg

    A. Stiehl

  2. Medizinische Universitätsklinik, Bergheimerstraße 58, 69115, Heidelberg

    A. Stiehl

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Correspondence to A. Stiehl.

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Stiehl, A. Primär sklerosierende Cholangitis. Internist 45, 27–32 (2004). https://doi.org/10.1007/s00108-003-1116-0

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  • DOI: https://doi.org/10.1007/s00108-003-1116-0

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