AbstractPrimary sclerosing cholangitis often occurs in association with inflammatory bowel disease, particularly ulcerative colitis but also Crohn's disease of the colon either with or without terminal ileal disease. Little data exist as to the effect of inflammatory bowel disease on the presenting symptoms, radiological features, response to liver transplantation, and potential risk of bile duct carcinoma in individuals with primary sclerosing cholangitis. In an effort to answer these questions, 66 patients with primary sclerosing cholangitis were studied. The definitive diagnosis of primary sclerosing cholangitis in each was accomplished using cholangiography, which in each case demonstrated characteristic beading, ectasia and stricturing of the intrahepatic and extrahepatic bile ducts.Inflammatory bowel disease was present in 47 (71.2%) patients. Thirty nine (59.1%) had ulcerative colitis; their mean age was 42.5 ± 11.6 yr (mean ±SD), and the male/female ratio was 2.9: 1. In addition, eight patients (12.1%) had Crohn's colitis; their mean age was 40.5 ± 6.5 yr, and the male/female ratio of this group was 1:1. Nineteen patients (28.8%) had primary sclerosing cholangitis without any inflammatory bowel disease; their mean age was 42.0 ± 12.1 yr, and the male/female ratio in this group was 0.72:1. Seventy‐two percent of the patients without inflammatory bowel disease had either jaundice, pruritus or fatigue at presentation compared with 41% of the patients with inflammatory bowel disease (p<0.05). In contrast, abnormal liver function tests were more common as the first manifestation of liver disease in the latter group (38% vs. 11%; p<0.05).Combined intrahepatic and extrahepatic bile duct involvement was found more frequently in patients with inflammatory bowel disease than in those without inflammatory bowel disease (81.5% vs. 46.2%, p<0.05). In contrast, involvement of the extrahepatic bile ducts alone was more frequent in patients without inflammatory bowel disease (38.4% vs. 7.4%, p<0.05). Thirty‐eight subjects (58%) underwent orthotopic liver transplantation. Two‐year survival after orthotopic liver transplantation for the groups with and without inflammatory bowel disease was 84% and 100%, respectively (NS).During this time, six patients with bile duct carcinoma were admitted. In four patients the diagnosis of bile duct carcinoma was established at time of referral and in two patients at liver transplantation. Five of these six patients had inflammatory bowel disease. The prevalence of bile duct carcinoma in patients with and without inflammatory bowel disease was similar (9.8% vs. 5%, respectively; NS). None of the transplanted patients have experienced a disease recurrence within a mean follow‐up period of 24.6 ± 11.8 mo.Based on these observations, it is concluded that primary sclerosing cholangits occurring in association with inflammatory bowel disease differs from primary sclerosing cholangitis occurring in the absence of inflammatory bowel disease differs from primary sclerosing cholangitis occurring in the absence of inflammatory bowel disease in terms of its location and gender distribution. (HEPATOLOGY 199