Primary Sclerosing Cholangitis

Question 1

Definition of PSC

Answer 1

• Intrahepatic and extrahepatic ducts become strictured and fibrotic
• This causes obstruction to flow of bile out of the liver and into the intestines
• Sclerosis refers to the stiffening and hardening of the bile ducts and cholangitis is inflammation
• Chronic bile obstruction can lead to hepatitis, fibrosis and cirrhosis

Question 2

Presentation of PSC

Answer 2

• Risk factors
  
  • Male
  • Aged 30-40 years
  • UC
  • FHx
• Presentation
  
  • Jaundice
  • Chronic RUQ pain
  • Pruritis
  • Fatigue
  • Hepatomegaly

Question 3

Diagnosis of PSC

Answer 3

• Cholestatic pitcure (raised ALP - most deranged LFT)
• pANCA, ANA and aCL
• MR cholangiopancreatography is gold standard

Question 4

Management of PSC

Answer 4

• ERCP to dilate and stent strictures
• Ursodeoxychlic acid to slow disease progression
• Colestyramine is a bile acid sequestrate
• Monitoring for compications