Primary Biliary Cirrhosis

Question 1

What is primary biliary cirrhosis?

Answer 1

Chronic inflammation and fibrous obliteration of intrahepatic bile ducts

Question 2

What is the aetiology/epidemiology of PBC?

Answer 2

• ?AI (a/w Sjogren’s syndrome, scleroderma, CREST, RA, thyroiditis).
• mainly middle aged women (1M:9F).

Question 3

Signs and symptoms of PBC?

Answer 3

• Pruritus, fatigue
• Chronic: jaundice, melanosis, other signs of cholestasis
• End stage: hepatocellular failure, portal HTN, ascites

Question 4

LFTs in PBC?

Answer 4

• Increased ALP and GGT

- Bilirubin rises in later stages

Question 5

Ix in PBC workup?

Answer 5

• LFTs
• Anti-mitochondrial Ab (95% sens and spec)
• Increased cholesterol
• Liver biopsy: confirmation and staging
• MRCP: rule out bile duct obstruction

Question 6

Treatment of PBC?

Answer 6

• Ursodiol (/colchicine, methotrexate)
• Cholestyramine
• Calcium and Vit D for low bone density
• Bisphosphonates if osteoporosis severe
• Monitor for thyroid disease
• Transplant if severe / progressive