Primary Biliary Cirrhosis

Question 1

Pathophysiology?

Answer 1

• immune system attacks small bile ducts in liver (intrahepatic ducts)–> obstruction of flow of bile
• liver usually excretes bile acids, billirubin & cholesterol. when there is obstruction to outflow of these chemicals, they get absorbed into bloodstream and buildup
• back pressure causes fibrosis, cirrhosis and liver failure

Question 2

aetiology?

Answer 2

Auto-immune

• Anti-mitochondrial antibodies
• ANA +ve

Question 3

Symptoms?

Answer 3

• fatigue
• abdo pain
• jaundice
• itching
  
  • due to build up of bile acids
• pale, greasy stools
  
  • pale= no billirubin and greasy=fat malabsorption
• xanthelasma
  
  • build up of cholesterol

Question 4

signs?

Answer 4

of cirrhosis and liver failure

Question 5

Ix?

Answer 5

• LFTs- ALP raised
• Autoantibodies
• IgM raised
• liver biopsy to diagnose & stage

Question 6

Mx?

Answer 6

• Ursodeoxycholic acid
  
  • reduces absorption of cholesterol
• Colestyramine
  
  • binds to bile to prevent absorption
• Immunosuppression
• Liver transplant

Question 7

complications?

Answer 7

• liver cirrhosis, HCC & portal HT
• Hypothyroidism (Abs against thyroid) & osteoporosis