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Primary sclerosing cholangitis.

J A Summerfield

    Postgraduate Medical Journal
    |January 1, 1983
    PubMed
    Summary
    This summary is machine-generated.

    Primary sclerosing cholangitis (PSC) is an increasingly recognized liver disease, often associated with inflammatory bowel disease. Its variable prognosis means it

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    Area of Science:

    • Hepatology
    • Gastroenterology
    • Immunology

    Background:

    • Primary sclerosing cholangitis (PSC) is increasingly diagnosed as a cause of liver disease.
    • Widespread cholangiography, particularly ERCP, aids in diagnosis.
    • PSC is associated with inflammatory bowel disease, notably ulcerative colitis (approx. 3% of UC patients).

    Purpose of the Study:

    • To review the current understanding of primary sclerosing cholangitis.
    • To discuss its varied clinical presentations and associations.
    • To evaluate the prognosis and current treatment limitations.

    Main Methods:

    • Review of clinical presentations and diagnostic findings in PSC.
    • Analysis of associations between PSC and other fibrosing diseases, especially inflammatory bowel disease.

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  • Evaluation of prognostic data and treatment outcomes.

    • Main Results:

    • Hepatic histology is diagnostic in a minority of patients.
    • Clinical presentations range from cholestatic illness and acute cholangitis to simulated chronic liver diseases or asymptomatic cases.
    • Prognosis is variable, with potential for long-term remission, challenging the view of invariably progressive disease.

    Conclusions:

    • Primary sclerosing cholangitis has diverse clinical manifestations and associations.
    • The disease course is variable, not always progressive.
    • Current treatments remain unsatisfactory.