Primary sclerosing cholangitis (PSC)

Question 1

1) What is PSC?
2) What does this cause?
3) The cause of PSC is a combination of what 4 factors?
4) There’s an established association with what other condition?

Answer 1

1) Condition where the intrahepatic or extrahepatic ducts become strictured and fibrotic
2) Obstruction to the flow of bile out of the liver and into the intestines
3) Genetic, autoimmune, intestinal microbiome and environmental factors
4) Ulcerative colitis

Question 2

Name 2 risk factors

Answer 2

• Male
• Aged 30-40
• Ulcerative Colitis
• Family History

Question 3

Name 2 ways it can present

Answer 3

• Jaundice
• Chronic right upper quadrant pain
• Pruritus
• Fatigue
• Hepatomegaly

Question 4

1) In the ‘cholestatic picture’ - what liver function marker is the most deranged?
2) What is the gold standard diagnostic investigation for PSC?

Answer 4

1) ALP (alkaline phosphatase)
2) Magnetic resonance cholangiopancreatography (MRCP)

Question 5

Name 2 complications of PCS

Answer 5

• Acute bacterial cholangitis
• Cholangiocarcinoma develops in 10-20% of cases
• Colorectal cancer
• Cirrhosis and liver failure
• Biliary strictures
• Fat soluble vitamin deficiencies