Primary sclerosing cholangitis

Question 1

What is primary sclerosing cholangitis?

Answer 1

Autoimmune condition in which intrahepatic/extrahepatic bile ducts become strictured and fibrotic

Question 2

There is a well established association between primary sclerosing cholangitis and which other autoimmune condition?

Answer 2

Ulcerative colitis

Question 3

What are the clinical features of primary sclerosing cholangitis?

Answer 3

Features of cholestasis:

• Pruritus (build up of bile acids)
• Jaundice
• Pale stools and dark urine

Question 4

What would the LFTs show (initially) in a patient with PBC?

What may the LFTs show as time progresses?

Answer 4

“Cholestatic”/obstructive picture:

• High alk phos
• High bilirubin

Over time, ALT/AST (transaminases) may also be raised as the disease progresses to hepatitis

Question 5

Which antibodies may be associated with PSC?

Answer 5

p-ANCA

Question 6

What is the gold standard diagnostic investigation for PSC?

Answer 6

MRCP

Question 7

Patients with PSC are at increased risk of developing…

Answer 7

• Acute cholangitis

- Cholangiocarcinoma

Question 8

Describe the medical and interventional management of PSC

Answer 8

Medical:

• Symptomatic: cholestyramine (pruritus)
• Disease modifying: ursodeoxycholic acid

Interventional:
- ERCP to stent/dilate strictures