PSC

Question 1

What is PSC?

Answer 1

progressive liver disease with inflammatory fibrosis + obliteration of intrahepatic + extrahepatic bile ducts

Question 2

Describe the aetiology of PSC

Answer 2

UNKNOWN
Possible immune + genetic predisposition with environmental triggers

Question 3

List 3 conditions associated with PSC

Answer 3

UC
Crohns (much less than UC)
HIV

Question 4

Explain the relationship between PSC and UC

Answer 4

80% of PSC pts have UC
4% UC pts have PSC

Question 5

Describe the epidemiology of PSC

Answer 5

M > F (2:1)
25-40y

Question 6

List 5 S/S of PSC

Answer 6

Pruritis
Jaundice
Pale stool, dark urine
RUQ pain
Fatigue

Question 7

List 3 late signs of PSC

Answer 7

Hepatosplenomegaly
Portal hypertension
Liver failure

Question 8

What should you look for in the PMH if PSC is suspected?

Answer 8

UC

Question 9

Bloods for PSC?

Answer 9

FBC
LFTs, Albumin
Lipid profile
Autoantibodies
Immunoglobulins

Question 10

What is seen on LFTs in PSC?

Answer 10

High ALP
High GGT
Norm/ high BR
Mild transaminitis/ norm ALT + AST

Question 11

Describe the serology found in PSC

Answer 11

pANCA (80%)
ANA (75%)
IgG high

Question 12

Why is imaging performed in PSC?

Answer 12

r/o biliary obstruction
confirm dx with MRCP

Question 13

Which is the confirmatory investigation of choice in PSC?

Answer 13

MRCP (if no biliary obstruction)

(ERCP if obstruction)

Question 14

What is seen on MRCP in PSC?

Answer 14

multifocal intrahepatic + extrahepatic bile duct strictures

Question 15

Why perform liver biopsy in PSC?

Answer 15

Not routinely performed in workup
Performed in small duct/ AI hepatitis overlap syndrome

Question 16

What may be seen on biopsy in PSC?

Answer 16

concentric periductal “onion skin” fibrosis

Question 17

Which ducts are affected in PSC?

Answer 17

INTRAHEPATIC

EXTRAHEPATIC

Question 18

Describe management of PSC

Answer 18

Mainly supportive
No medications prevent progression

Question 19

Describe pharmacological management of PSC

Answer 19

Ursodeoxycholic acid

Question 20

What interventional management may be performed in PSC? What are the indications for this?

Answer 20

ERCP dilation +/- stenting
To relieve Sx caused by stenosis, e.g., pruritus +/or cholangitis
To exclude cholangiocarcinoma

Question 21

List 4 complications of PSC

Answer 21

Steatorrhea + fat soluble vitamin deficiency
Cirrhosis
Bacterial cholangitis
Malignancy

Question 22

Which malignancies may arise in PSC?

Answer 22

Cholangiocarcinoma
Gallbladder carcinoma
Colorectal cancer

Screen annually with abdo US/ MRI + colonoscopy

Question 23

What is the curative treatment for PSC?

Answer 23

Liver transplant, but high rate recurrence