Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + • Autoimmune disease characterized by portal tract inflammation +++ Symptoms and Signs + • Gradual increasing fatigue and pruritus +++ Laboratory Findings + • Gradual hyperbilirubinemia often evolving over 20 years +++ Rule Out + • Biliary obstruction secondary to anatomic lesion + • History and physical exam• CT abdomen• ERCP• Liver function tests• C-reactive protein levels• Anti-smooth muscle cell antibody• Anti-mitochondrial antibody• Liver biopsy +++ Surgery + • Liver transplantation +++ Indications + • Bilirubin > 10 +++ Contraindications + • Medical comorbidity, active malignancy +++ Treatment Monitoring + • Bilirubin level +++ Complications + • Primary nonfunction• Rejection• Biliary leak or stricture• Hemorrhage• Hepatic artery thrombosis +++ Prognosis + • 60-70% 5-year survival following liver transplant +++ References ++Talwalkar JA, Lindor KD. Primary biliary cirrhosis. Lancet. 2003;362:53. [PubMed: 12853201] Your MyAccess 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 Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth