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Sklerosierende Cholangitiden

Primäre, autoimmune und sekundäre Erkrankungen

Sclerosing cholangitis

Primary, autoimmune and secondary sclerosing diseases

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Zusammenfassung

Sklerosierende Cholangitiden sind als entzündlich-fibrosierende Erkrankungen der Gallenwege mit einem typischen „perlschnurartigen“ Bild im Cholangiogramm definiert. Die Ursachen können dabei mannigfaltig sein, so dass letztendlich eine Reihe unterschiedlicher Ätiologien in einem morphologisch ähnlichen Phänotyp münden. Die korrekte Diagnose ist jedoch in Hinblick auf Komplikationen und Therapie essenziell. Bei der primär sklerosierenden Cholangitis herrscht ein hohes Kolon- und Cholangiokarzinomrisiko vor, was für die anderen Entitäten nicht gezeigt ist. Ebenso ist die Assoziation mit chronisch-entzündlichen Darmerkrankungen verschieden. Therapeutisch profitieren Patienten mit IgG4-assoziierter Cholangitis von einer Steroidbehandlung, während Steroide bei primär und sekundär sklerosierender Cholangitis ineffektiv sind.

Abstract

Sclerosing cholangitis is a chronic cholestatic biliary disease leading to biliary strictures. Primary sclerosing cholangitis (PSC), IgG4-associated cholangitis (IAC) and secondary sclerosing cholangitis (SCC) continue to pose differential diagnostic difficulties. However, the correct diagnosis is crucial regarding differences in complications and therapeutic strategy. In contrast to IAC and SSC, PSC carries a high risk of cholangiocellular and colon cancer. Moreover, only PSC is associated with inflammatory bowel disease. Patients with IAC benefit from steroid therapy, whereas steroids are not effective in patients with SSC and PSC.

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Authors and Affiliations

  1. Medizinische Klinik und Poliklinik I, Allgemeine Innere Medizin mit den Schwerpunkten Gastroenterologie, Hepatologie, Nephrologie, Infektiologie, Endokrinologie und Diabetologie, Universitätsklinikum Bonn, Bonn, Deutschland

    T.J. Weismüller & C.P. Strassburg

  2. Klinik für Gastroenterologie, Hepatologie und Endokrinologie, Medizinische Hochschule Hannover, Carl Neuberg Str. 1, 30655, Hannover, Deutschland

    C.P. Strassburg, M.P. Manns &  T.O. Lankisch

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  1. T.J. Weismüller
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  2. C.P. Strassburg
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  3. M.P. Manns
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  4. T.O. Lankisch
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Correspondence to T.O. Lankisch.

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Weismüller, T., Strassburg, C., Manns, M. et al. Sklerosierende Cholangitiden. Gastroenterologe 7, 483–492 (2012). https://doi.org/10.1007/s11377-011-0623-0

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  • DOI: https://doi.org/10.1007/s11377-011-0623-0

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