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Semin Liver Dis 2006; 26(1): 022-030
DOI: 10.1055/s-2006-933560
DOI: 10.1055/s-2006-933560
Primary Sclerosing Cholangitis: Epidemiology, Natural History, and Prognosis
Further Information
Publication History
Publication Date:
23 February 2006 (online)
ABSTRACT
Primary sclerosing cholangitis is a progressive disease associated with significant morbidity and mortality that continues to draw attention from investigators worldwide. Epidemiologic studies indicate that its annual incidence remains stable, although more asymptomatic cases are being diagnosed. Nevertheless, even asymptomatic cases can progress to end-stage liver disease, albeit at a highly variable rate. Moreover, patients are predisposed to several malignancies, especially cholangiocarcinoma, which contributes to their decreased survival. On the other hand, a subgroup of patients with the so-called small-duct variant may have a better prognosis, and those with overlap syndromes with autoimmune hepatitis may respond well to immunosuppressants. Otherwise, efficient treatment is not available for patients with primary sclerosing cholangitis. Thus, even though it occurs rarely, this disease is commonly an indication for liver transplantation. Fortunately, transplantation outcomes are excellent, with overall 5-year survival rates of approximately 80%.
KEYWORDS
Primary sclerosing cholangitis - epidemiology - natural history - liver transplantation
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Keith D LindorM.D.
Division of Gastroenterology and Hepatology, Mayo Foundation
200 1st Street SW, Rochester, MN 55905
Email: lindor.keith@mayo.edu