Primary sclerosing cholangitis: a heterogenous disease.

The clinical, radiologic and pathologic features of 37 patients diagnosed as having primary sclerosing cholangitis (PSC) were reviewed. Sixty-two per cent were men, and 35% had ulcerative colitis. The patients demonstrated considerable variability in their natural history and pathology. It appeared that they could be divided into four fairly distant groups (1) sclerosing cholangitis affecting primarily the distal common bile duct; (2) sclerosing cholangitis occurring soon after an attack of acute necrotizing cholangitis; (3) chronic diffuse sclerosing cholangitis; and (4) chronic diffuse sclerosing cholangitis associated with inflammatory bowel disease. It is suggested that the patients in these groups may have different etiologies, may respond to different treatment regimes, and may have different prognoses.