Patients with small duct primary sclerosing cholangitis have a favourable long term prognosis

• Published in: Gut Vol. 51; no. 5; pp. 731 - 735
• Main Authors: Björnsson, E, Boberg, K M, Cullen, S, Fleming, K, Clausen, O P, Fausa, O, Schrumpf, E, Chapman, R W
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd and British Society of Gastroenterology 01.11.2002; BMJ; BMJ Publishing Group Ltd; BMJ Publishing Group LTD; Copyright 2002 by Gut
• Subjects: Abdomen; Adult; Age; alanine aminotransferase; alkaline phosphatase; ALP; ALT; aspartate aminotransferase; AST; Bile; Bile Duct Neoplasms - pathology; Bile Ducts - pathology; Bile Ducts - physiopathology; Biological and medical sciences; cholangiocarcinoma; Cholangiocarcinoma - pathology; Cholangitis; Cholangitis, Sclerosing - mortality; Cholangitis, Sclerosing - pathology; Cholangitis, Sclerosing - physiopathology; Crohn’s disease; Disease Progression; endoscopic retrograde cholangiopancreatography; ERCP; Female; Follow-Up Studies; Gallbladder diseases; Gastroenterology. Liver. Pancreas. Abdomen; Hepatitis; Humans; IBD; Inflammatory bowel disease; INR; international normalised ratio; LFTs; Liver cirrhosis; Liver Disease; Liver diseases; Liver Function Tests; liver transplantation; Liver. Biliary tract. Portal circulation. Exocrine pancreas; Male; Medical prognosis; Medical sciences; Middle Aged; Mortality; Norway; Other diseases. Semiology; Pain; Patient outcomes; primary sclerosing cholangitis; Prognosis; PSC; Risk; Sweden; ulcerative colitis; United Kingdom; Norway; United Kingdom; Sweden; Human; Small dimension; Prognosis; Hepatic disease; Malignant tumor; Biliary tract disease; Bile canaliculi; Statistical study; Survival; Incidence; Primitive; Malignant cholangioma; Digestive diseases; Complication; Lesion; Sclerosing cholangitis
• ISSN: 0017-5749; 1468-3288; 1458-3288
• DOI: 10.1136/gut.51.5.731

Background: Patients with cholestatic liver function tests and histological features of primary sclerosing cholangitis (PSC) but a normal cholangiogram are considered to have small duct PSC. The natural history of this condition is unknown. Methods: Thirty three patients with small duct PSC were identified among patients admitted for diagnostic workup of cholestatic liver function tests in one centre in the UK (Oxford) and one centre in Norway (Oslo). A total of 260 patients with large duct PSC were compared, and prognosis in terms of death, cholangiocarcinoma, biochemical features, histological features, and symptoms analysed. Results: Mean age at diagnosis was 38 years and 39 years in small duct and large duct PSC, respectively. Mean follow up was 106 months in small duct and 105 months in large duct patients. Four patients originally considered to have small duct developed large duct PSC. Two of these underwent liver transplantation during follow up. Of the remainder who did not develop large duct PSC, two patients died during follow up: one of liver failure and the other of cardiac death unrelated to her liver disease. A total of 122 (47%) large duct patients either required liver transplantation (34 patients) or died (88 patients). Small duct patients had a significantly better survival compared with large duct patients. Among small duct patients, none developed cholangiocarcinoma compared with 28 of 260 (11%) large duct patients. Conclusions: Patients with small duct PSC seem to have a good prognosis in terms of survival and development of cholangiocarcinoma. Small duct PSC progresses to large duct PSC in a small proportion of patients.