Cirrhosis, Primary Biliary

• • 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