Primary Sclerosing Cholangitis (PSC)

Question 1

What does PSC stand for?

Answer 1

Primary Sclerosing Cholangitis?

Question 2

What is Primary Sclerosing Cholangitis?

Answer 2

Autoimmune destruction of intralobular and extralobular hepatic ducts (same pathology as PBC just affects more ducts)

Question 3

What are risk factors for PSC?

Answer 3

Male
40-50
Strong link to IBD (especially UC)

Question 4

What are symptoms of PSC?

Answer 4

Initially asymptomatic
Then pruritis and fatigue
Jaundice
Charcot triad if common bile duct involved
Hepatosplenomegaly
IBD

Question 5

What does Charcot’s triad consist of?

Answer 5

Jaundice
ULQ pain
Fever

Question 6

How is PSC diagnosed?

Answer 6

Serology:
Check for acute hepatitis infection - HBVsAg and HCVAb

AMA negative (unlike PBC)
Coeliac screen: anti tTG
pANCA positive (33-88%)

Gold standard imaging = MRCP
(magnetic resonance cholangiopancreatography)

Question 7

Does ursodeoxycholic acid (lifetime) work for PSC?

Answer 7

No, only PBC

Question 8

What is treatment for PSC?

Answer 8

Conservative symptom management:
Colestyramine for pruritis
Fat soluble ADEK supplements

Consider liver transplant