Primary sclerosing cholangitis

Two new cases of primary sclerosing cholangitis with a rapid fatal course, particularly the patient in Case 2, are presented. Primary sclerosing cholangitis is a rare condition of unknown cause presenting with extrahepatic biliary obstruction due to a chronic inflammatory obliterative process with the absence of stones, cancer, or previous biliary surgery. This condition is often associated with ulcerative colitis, retroperitoneal fibrosis, and Riedel's thyroiditis. Surgical treatment for promoting bile drainage and long-term corticosteroid therapy are effective palliative measures.