Primary Sclerosing Cholangitis

Question 1

What is PSC?

Answer 1

A chronic cholestatic disorder characterised by inflammation and fibrosis of intrahepatic and extraheptic bile ducts causing multifocal strictures

Question 2

70% of patients also have what?

Answer 2

Ulcerative colitis

Question 3

Chronic bile duct obstruction eventually leads to what?

Answer 3

Hepatitis, fibrosis & cirrhosis

Question 4

Risk factors?

Answer 4

(1) Male
(2) UC
(3) aged 30-40
(4) FHx

Question 5

Presentation?

Answer 5

• RUQ pain (chronic)
• jaundice & hepatomegaly
• pruritis
• fatigue, weight loss

Question 6

LFT result?

Answer 6

ALP rises first (cholestatic picture)

Others deranged as progresses

Question 7

What antibodies may be present?

Answer 7

• ANA (anti-nuclear antibodies)
• p-ANCA (anti-neutrophil cytoplasmic antibody)
• aCL (Anticardiolipin antibody)
• Anti-Smooth muscle

Question 8

Gold standard investigation for diagnosis?

Answer 8

MRCP - Magnetic resonance cholangiopancreatography

Question 9

What does MRCP show?

Answer 9

Multiple beaded biliary strictures

Question 10

Which cancers are PSC patients more at risk of?

Answer 10

cholangiocarcinoma (10-20%)

colorectal cancer

Question 11

Associations & complications?

Answer 11

(1) Acute bacterial cholangitis
(2) Cholangiocarcinoma
(3) Colorectal cancer
(4) Cirrhosis/liver failure/ oesophageal varices
(5) biliary strictures
(6) fat soluble vitamin deficiencies

Question 12

Management?

Answer 12

(1) ERCP - dilate strictures
(2) Ursodeoxycholic acid
(3) cholestyramine - itch
(3) Supplement ADEK
- -> liver transplant