Primary Biliary Cirrhosis

Question 1

Define primary biliary cirrhosis

Answer 1

Chronic inflammatory liver disease involving the progressive destruction of intrahepatic bile
ducts -> cholestasis -> cirrhosis

Question 2

What are the causes/risk factors of primary biliary cirrhosis?

Answer 2

Unknown aetiology
Environmental trigger (infection, chemical, toxin) -> T-cell mediated autoimmune response
against bile duct epithelial cells in genetically susceptible patients

Risk factors
• Female (9:1)
• Autoimmune disease e.g. Sjorgen’s (keratoconjunctivitis sicca), scleroderma, thyroid
disease, rheumatoid arthritis

Question 3

What are the symptoms of primary biliary cirrhosis?

Answer 3

• Asymptomatic
• Pruritus
• Fatigue
• Jaundice

Question 4

What are the signs of primary biliary cirrhosis?

Answer 4

• Jaundice
• Hepatomegaly
• Xanthelasma, xanthoma

Question 5

What investigations are carried out for primary biliary cirrhosis?

Answer 5

• Raised ALP and GGT
• Raised cholesterol
• Anti-mitochondrial antibodies (present
in >95%)
• Elevated IgG and IgM
• Prolonged PT due to reduced absorption of vitamin K