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  • • 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]

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