Primary biliary cholangitis

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

• Ursodeoxycholic acid