Primary sclerosing cholangitis is a rare syndrome of unknown cause, chronic insidious course, and very difficult management. It is characterized by severe chronic inflammation and fibrosis of the intrahepatic and extrahepatic biliary ducts. Treatment consists of prolonged choledochal drainage, antibiotics, and corticosteroid therapy. The criteria for making this diagnosis are (1) progressive obstructive jaundice; (2) absence of cholelithiasis; (3) absence of previous biliary surgery (4) absence of primary biliary cirrhosis on biopsy; (5) absence of biliary tract malignant disease on long-term, follow-up; (6) absence of associated diseases such as ulcerative colitis, retroperitoneal fibrosis, regional enteritis, chronic thyroiditis; (7) general-ized involvement of the biliary duct, with fibrosis and stenosis.
One patient with primary sclerosing cholangitis confirmed by clinically and histologic examination were presented with a brief review of literatures.
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