Primary Sclerosing Cholangitis

Question 1

Define primary sclerosing cholangitis

Answer 1

Chronic cholestastic liver disease characterised by progressive inflammatory fibrosis and
obliteration of intrahepatic and extrahepatic bile ducts -> diffuse multi-focal stricture
formation

Question 2

What are the causes/risk factors for primary sclerosing cholangitis?

Answer 2

Unknown aetiology
Genetic susceptibility (HLA haplotypes and p-ANCA)
Risk factors
• Male (2:1)
• Ulcerative colitis (70% of PSC patients have UC, 5% of UC patients will develop PSC)

associated with cholangiocarcinoma

Question 3

What are the symptoms of primary sclerosing cholangitis?

Answer 3

• Asymptomatic
• Intermittent jaundice
• Intermittent pruritus
• RUQ pain
• Weight loss
• Fatigue
• Episodic cholangitis
- RUQ pain
- Fever
- Jaundice

Question 4

What are the signs of primary sclerosing cholangitis?

Answer 4

• Jaundice

Question 5

What investigations are carried out for primary sclerosing cholangitis?

Answer 5

• Raised ALP, ALT,
• Raised conjugated BR
• Prolonged PT
• Raised IgG in children and IgM in adults
• Raised cholesterol
• p-ANCA present
• ASM and ANA may be present (AMA mainly absent to exclude PBC)
• Liver biopsy to confim diagnosis