Primary sclerosing cholangitis is a chronic cholestatic liver disease of unknown etiology that leads to progressive obliterative fibrosis of the biliary tree, ultimately progressing to death from liver failure or cholangiocarcinoma. Immune-mediated factors are thought to be involved in its pathogenesis, as suggested by the presence of circulating antineutrophil cytoplasmic antibodies, mainly IgG1 and lgG3. This test is not yet useful as a diagnostic tool because sensitivity and specificity vary widely according to the different detection methods used. A small subset of T cells bearing the Tδ receptor may also be involved in its pathogenesis. Cigarette smoking appears to be protective. No medical treatment is of proven benefit, and median survival from diagnosis is 12 years. Ursodeoxycholic acid was considered a promising drug, but no important benefits were demonstrated in a large, randomized, controlled trial. In selected patients endoscopic treatment of major extrahepatic strictures may be tried. Orthotopic liver transplantation remains the only effective treatment, but the disease may recur in some patients.
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