RESUMO
Primary sclerosing cholangitis is a chronic cholestatic disease that may ultimately progress to hepatic parenchymal dysfunction and death from premature liver failure. Symptomatic patients should undergo radiological evaluation to exclude secondary causes of cholangitis and to evaluate the extent and location of biliary ductal disease. A percutaneous liver biopsy is advisable in any patient with clinical or biochemical evidence of cirrhosis. Patients with diffuse ductal involvement should initially receive medical treatment, preferably in a center conducting prospective clinical trials. Liver transplantation should be considered the initial procedure in primary sclerosing cholangitis patients with diffuse ductal involvement, complications of cirrhosis or deteriorating liver function. Nontransplant procedures should be restricted to symptomatic patients with a dominant extrahepatic stricture. In properly selected patients, effective and durable palliation of symptoms can be anticipated with biliary enteric drainage. Many currently available techniques obviate the need for longterm transanastomotic stenting, thus minimizing the risk of recurrent cholangitis and the need for repeated tube changes. These patients may also be candidates for percutaneous or endoscopic balloon cholangioplasty. Liver transplantation should also be considered in patients with a dominant stricture and histologic evidence of biliary cirrhosis or hepatic deterioration following a nontransplant procedure.