Primary Sclerosing Cholangitis

Abstract

The clinical, radiologic and pathologic features of 37 patients diagnosed as having primary sclerosing cholangitis (PSC) were reviewed. Of these, 62% were men, and 35% had ulcerative colitis. The patients demonstrated considerable variability in their natural history and pathology. They could be divided into 4 fairly distinct groups: sclerosing cholangitis affecting primarily the distal common bile duct; sclerosing cholangitis occurring soon after an attack of acute necrotizing cholangitis; chronic diffuse sclerosing cholangitis; and chronic diffuse sclerosing cholangitis associated with inflammatory bowel disease. The patients in these groups may have different etiologies, may respond to different treatment regimes and may have different prognoses.