Primary Sclerosing Cholangitis

Question 1

This is an autoimmune destructive disease of ___ and ___ sized bile ducts.
within and outwith the liver

Answer 1

This is an autoimmune destructive disease of large and medium sized bile ducts.

Question 2

pathology: Chronic inflammatory process affecting the intra-and extra-hepatic bile ducts which leads to periductal ____, duct destruction, _____ and _____

Answer 2

Chronic inflammatory process affecting the intra-and extra-hepatic bile ducts which leads to periductal fibrosis, duct destruction, jaundice and fibrosis

Question 3

it is associated with ___

Answer 3

UC

Question 4

Increased risk of malignancy in the ____ ___ and ___- (note PSC and cirrhosis more associated with carcinoma of the liver)

Answer 4

Increased risk of malignancy in the bile ducts and colon (note PSC and cirrhosis more associated with carcinoma of the liver)

Question 5

___ -____ fibrosis seen histologically

Answer 5

onion skinning

Question 6

more common in males

Answer 6

yes

Question 7

what are the symptoms?

Answer 7

prutitus and fatigue

Question 8

how is it diagnosed?

Answer 8

Increased Alk phos, then increased bilirubin and hypergammaglobulinaemia

Liver biopsy: shows a fibrous, obliterative cholangitis