Primary Sclerosing Cholangitis (PSC)

Question 1

What is primary sclerosing cholangitis (PSC)?

Answer 1

A biliary disease of unknown aetiology characterised by inflammation and fibrosis of intra and extra-hepatic bile ducts

Common in men and associated with ulcerative colitis (UC)

Question 2

WHO is PSC mostly commonly associated with?

Answer 2

• Men
• PMHx: UC

Question 3

What percentage of patients with ulcerative colitis have PSC?

Answer 3

4%

80% of patients with PSC have ulcerative colitis

Question 4

What are common associations with primary sclerosing cholangitis?

Answer 4

• Ulcerative colitis
• Crohn’s disease (much less common association)
• HIV

Question 5

What is cholestasis?

Answer 5

A condition where the flow of bile from the liver to the duodenum is impaired

Question 6

List common features of primary sclerosing cholangitis.

Answer 6

• Cholestasis
• RUQ pain
• Jaundice
• Pruritus
• Raised bilirubin
• Raised ALP
• Fatigue

Question 7

What are the standard diagnostic investigations for PSC?

Answer 7

• Endoscopic retrograde cholangiopancreatography (ERCP)
• Magnetic resonance cholangiopancreatography (MRCP)

Question 8

What does ERCP or MRCP show in cases of PSC?

Answer 8

Multiple biliary strictures giving a ‘beaded’ appearance

Question 9

What autoantibody may be present in PSC?

Answer 9

p-ANCA

Question 10

What might liver biopsy show in PSC?

Answer 10

Fibrous, obliterative cholangitis often described as ‘onion skin’

Question 11

What is the first line management for PSC?

Answer 11

• ** Ursodeoxycholic acid**
• Supportive care (fat-soluble vitamin supplementation, regular screening for cholangiocarcinoma, Vit D)
• ** Cholestyramine **for pruritus

Question 12

What are the surgical options for managing PSC?

Answer 12

• ERCP with stenting
• Liver transplant (complete cure!)

Question 13

What are potential complications of primary sclerosing cholangitis?

Answer 13

• Cholangiocarcinoma (in 10%)
• Increased risk of colorectal cancer