Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + • Associated with ulcerative colitis 40-60%, pancreatitis 12-25%, diabetes mellitus 5-10%, and rarely other autoimmune disorders• Onset during fourth or fifth decade of life• Increased risk for cholangiocarcinoma +++ Symptoms and Signs + • Intermittent jaundice• Fever• Right upper quadrant pain• Pruritus +++ Laboratory Findings + • Elevated alkaline phosphatase• Hyperbilirubinemia• Leukocytosis +++ Imaging Findings + • Right upper quadrant US, ERCP, and magnetic resonance cholangiopancreatography (MRCP) may show multiple dilatations and strictures of extrahepatic biliary ducts + • Cholangiocarcinoma• Presence of cirrhosis +++ Rule Out + • Cholangiocarcinoma + • History and physical exam• Liver function tests• Abdominal US• ERCP• Liver biopsy if question of cirrhosis• Brushings by percutaneous transhepatic cholangiography (PTC) or ERCP if question of malignancy +++ When to Admit + • Cholangitis +++ Surgery + • Balloon dilatation of multiple strictures• Resection of dominant stricture followed by biliary reconstruction• Liver transplantation (preferably before onset of cirrhosis) +++ Contraindications + • Cirrhosis +++ Medications + • Ursodiol (improves liver function and histology but no difference in 5-year clinical outcome) +++ Treatment Monitoring + • Alkaline phosphatase levels +++ Complications + • Cholangitis• Recurrent strictures• Primary nonfunction• Allograft rejection• Recurrent stricture post-transplant +++ Prognosis + • 85% survival rate 5 years post transplant• 71% actuarial survival at 5 years for resection of dominant stricture (only 20% if cirrhosis present)• 43% long-term success with balloon therapy for multiple strictures +++ References ++Kim WR et al. A revised natural history model for primary sclerosing cholangitis. Mayo Clin Proc. 2000;75:688. [PubMed: 10907383] ++van Hoogstraten HJ et al. Ursodeoxycholic acid therapy for primary sclerosing cholangitis: results of a 2-year randomized controlled trial to evaluate single versus multiple daily doses. J Hepatol. 1998;29:417. ++Kubicka S et al: K-ras mutations in the bile of patients with primary sclerosing cholangitis. Gut 2001;48:403. [PubMed: 11171833] ++Ryder SD, Beckingham IJ: ABC of diseases of liver, pancreas, and biliary system. Other causes of parenchymal liver disease. BMJ 2001;322:290. [PubMed: 11157536] Your Access profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Password? Forgot Username? Sign in via OpenAthens Sign in via Shibboleth