Primary biliary cholangitis/cirrhosis

Question 1

Primary biliary cholangitis/cirrhosis: Definition

Answer 1

damage to heptic interlobular bile ducts due to chronic hepatic inflammation. It can progress to cirrhosis.

Question 2

Primary biliary cholangitis: Aetiology/associations

Answer 2

aetiology is unknown. Thought to be an autoimmune condition.
- Sjogren’s syndrome (seen in up to 80% of patients)
- rheumatoid arthritis
- systemic sclerosis
- thyroid disease

Question 3

Primary biliary cholangitis: Symptoms

Answer 3

classic presentation middle-aged woman
* asymptomatic
* RUQ pain (10%)
* fatigue
* cholestatic jaundice
* hyperpigmentation (esp. over pressure areas)
* xanthelasmas, xanthomata, clubbing, hepatosplenomegaly
* liver failure if late

Question 4

Primary biliary cholangitis: Investigations

Answer 4

• US or MRCP
• raised serum igM
  immunology
• Anti-mitochondrial antibodies (AMA) M2 subtype - highly specific and most common
• Smooth muscle antibodies

Question 5

Primary biliary cholangitis: Management

Answer 5

• first-line: ursodeoxycholic acid + reassessment of liver biochem at 1 year
  slows disease progression and improves symptoms
• pruritus: cholestyramine
• fat-soluble vitamin supplementation
• liver transplantation
  e.g. if bilirubin > 100 (PBC is a major indication)
  recurrence in graft can occur but is not usually a problem

Question 6

Primary biliary cholangitis: Complications

Answer 6

cirrhosis → portal hypertension → ascites, variceal haemorrhage
osteomalacia and osteoporosis
significantly increased risk of hepatocellular carcinoma (20-fold increased risk)

Question 7

Primary biliary cholangitis: How to remember

Answer 7

Primary biliary cholangitis - the M rule
IgM
anti-Mitochondrial antibodies, M2 subtype
Middle aged females