primary sclerosing cholitis

Question 1

definition

Answer 1

condition where the intrahepatic and extrahepatic ducts become fibrotic and strictured

Question 2

risk factors

Answer 2

male
30-40
UC
family history

Question 3

pathology

Answer 3

• non-specific ANACA antibodies attack bile ducts, causing multiple strictures
• chronic obstruction of bile causes a backpressure of bile onto the liver
• causing hepatitis - fibrosis - cirrhosis

Question 4

presentation

Answer 4

• jaundice
• fever
• pruritis
• RUQ pain
• fatigue
• hepatomegaly

Question 5

signs and symptoms

Answer 5

50% asymptomatic
- fatigue
- fever
- jaundice
- vitamin deficinecy
- pruritis
- upper abdominal pain
- cirrhosis

Question 6

investigations

Answer 6

• GGT elevated
• 1st line - abdo USS
• gold standard - MRCP - bead on a string appearance due to fibrosis in some places and not others

ANCA antibodies elevated in 94%
ANA antibodies elevated in 77%

Question 7

management

Answer 7

antipruritics
surgical balloon dilation
ERCP procedure
liver transplant

Question 8

complications

Answer 8

• acute bacterial cholangitis (bile duct infection)
• cholangiocarcinoma (cancer in bile duct)
• colorectal cancer
• cirrhosis and liver failure
• deficient fat soluble vitamins bc no bile, no digestion