Primary Sclerosing Cholangitis

Question 1

Aetiologies?

Answer 1

Closely associated with UC

Also HIV infection

Question 2

Pathology?

Answer 2

Diffuse chronic inflammation of the biliary tree, beginning lower down near sphincter of Oddi
Gradual obliteration of biliary tree

Question 3

Signs and symptoms?

Answer 3

Incidental finding in UC patients usually due to deranged LFT
Fatigue, intermittent jaundice, weight loss, RUQ pain, pruritus
Hepato/splenomegaly, signs of portal hypertension

Question 4

Investigations and Findings?

Answer 4

LFTs - increased ALP, increased bilirubin, in GGT

MRCP > ERCP - reveals stricture and small vs large duct disease

Question 5

Management?

Answer 5

Symptomatic relief
Preventing complications
Transplantation in younger patients

Question 6

Complications?

Answer 6

Increase risk of cholangiocarcinoma with slow progression into cirrhosis