SCLEROSING CHOLANGITIS

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 invol ved in its pathogenesis, as suggested by the presence of circulating a ntineutrophil cytoplasmic antibodies, mainly IgG(1) and IgG(3). This t est is not yet useful as a diagnostic tool because sensitivity and spe cificity vary widely according to the different detection methods used . A small subset of T cells bearing the gamma-delta receptor may also be involved in its pathogenesis. Cigarette smoking appears to be prote ctive. No medical treatment is of proven benefit, and median survival from diagnosis is 12 years. Ursodeoxycholic acid was considered a prom ising drug, but no important benefits were demonstrated in a large, ra ndomized, controlled trial. In selected patients endoscopic treatment of major extrahepatic strictures may be tried. Orthotopic liver transp lantation remains the only effective treatment, but the disease may re cur in some patients.