PBC

• Primary biliary cholangitis is an autoimmune condition where the immune system attacks the small intrahepatic bile ducts - resulting in obstructive jaundice and liver disease
• In primary biliary cholangitis cholestasis occurs, leading to increased bile acids, bilirubin and cholesterol build up in the blood
• A typical patient with primary biliary cholangitis is a white woman aged 40-60 years old. Most patients have anti-mitochondrial antibodies.
• Liver biopsy is used to diagnose primary biliary cholangitis
• The most common presenting features of PBC are fatigue and pruritus
• The first line treatment for PBC is ursodeoxycholic acid
• An estimated 50-60% of patients with PBC are asymptomatic and identified by abnormalities in liver function tests. The most commonly observed symptoms when present are pruritus and fatigue.

  • Fatigue
  • Pruritus
  • RUQ pain
  • Features of chronic liver disease
• Signs on examination:

  • Hepatomegaly
  • Excoriation marks
  • Xanthomas and Xanthelasmas
  • Dry skin
  • Hyperpigmentation
  • Jaundice
  • Features of chronic liver disease
• Diagnosis of PBC is based on the presence of cholestatic liver enzymes (ALP) and positive anti-mitochondrial antibodies.
• PBC is a slowly progressive disease that can lead to cirrhosis - risk of bleeding and hepatocellular carcinoma

  • Hypercholesterolaemia due to a decrease in functional low-density lipoprotein
  • Malabsorption of fat soluble vitamins (A,D,E,K)
  • Malabsorption of fat
  • Osteoporosis
• PBC will lead to positive IgM autoantibodies whereas autoimmune hepatitis will lead to positive IgG autoantibodies