Primary biliary cholangitis

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Clinical Manifestations

  • Fatigue and pruritus
  • Jaundice
  • Hepatosplenomegaly

Investigations

  • ALP always elevated (often very high), ALT may be mildly elevated
  • Antimitochondrial antibodies are 95% sensitive
  • ANA elevated in about 70%

Diagnosis

  • No extrahepatic biliary obstruction, no comorbidities affecting the liver, and at least two of the following three criteria:
    • ALP greater than 1.5 times the upper limit of normal
    • Antimitochondrial antibody titre of at least 1:40
      • Or sp100 or gp210 antibodies, if AMA negative
    • Histologic evidence of BC showing nonsuppurative destructive cholangitis and destruction of interlobular bile ducts

Management