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  • • Autoimmune disease characterized by portal tract inflammation

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Symptoms and Signs

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  • • Gradual increasing fatigue and pruritus

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Laboratory Findings

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  • • Gradual hyperbilirubinemia often evolving over 20 years

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Rule Out

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  • • Biliary obstruction secondary to anatomic lesion

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  • • History and physical exam

    • CT abdomen

    • ERCP

    • Liver function tests

    • C-reactive protein levels

    • Anti-smooth muscle cell antibody

    • Anti-mitochondrial antibody

    • Liver biopsy

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Surgery

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  • • Liver transplantation

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Indications

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  • • Bilirubin > 10

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Contraindications

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  • • Medical comorbidity, active malignancy

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Treatment Monitoring

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  • • Bilirubin level

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Complications

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  • • Primary nonfunction

    • Rejection

    • Biliary leak or stricture

    • Hemorrhage

    • Hepatic artery thrombosis

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Prognosis

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  • • 60-70% 5-year survival following liver transplant

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References

Talwalkar JA, Lindor KD. Primary biliary cirrhosis. Lancet. 2003;362:53.  [PubMed: 12853201]

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