Primary Biliary Cholangitis

Autoimmune disease of the liver which causes the destruction of the small bile ducts - liver only

Aetiology

  • Autoimmune
  • Much more common in women (10:1), especially middle-aged women

Pathophysiology

  • 90% of people with PBC have anti-mitochondrial antibodies (AMAs) against pyruvate dehydrogenase complex (PDC-E2) - enzyme complex found in mitochondria
  • T cell mediated - CD4+ cells react against antibodies

Clinical presentation

  • Usually asymptomatic/incidental finding - most will not develop symptoms, and most will not develop cirrhosis/liver failure
  • Symptoms can include:
    • Fatigue
    • Itch without rash
    • Xanthelasma and xanthomas

Investigations

2 of:
  • Positive AMA
  • Cholestatic LFTs - ALP > ALT/AST, ↑ GGT, ↑ bilirubin
  • Liver biopsy

Management

Ursodeoxycholic acid

  • First line
  • Increases flow of bile to wash the toxic bile salts out

Obeticholic acid

  • Second line
  • Alters the inflammatory cholesterol metabolism