primary sclerosis cholangitis

Question 1

define primary sclerosis cholangitis?

Answer 1

a chronic cholestatic liver disease characterised by progressive inflammation and fibrosis of intrahepatic and extrahepatic bile ducts

Question 2

cause of primary sclerosis cholangitis?

Answer 2

• unknown

- Possible immune and genetic predisposition

Question 3

most important association of primary sclerosis cholangitis?

Answer 3

• Ulcerative colitis

80% of patients with PSC have UC

Question 4

epidemiology of primary sclerosis cholangitis?

Answer 4

• Usually presents between 25-40 years of age

Question 5

presenting symptoms of primary sclerosis cholangitis?

Answer 5

•	May present with: 
•	Cholestasis 
o	Intermittent jaundice  
o	Pruritis  
o	Raised BR and ALP 
•	RUQ pain  
•	Weight loss  
•	Fatigue

Question 6

signs of primary sclerosis cholangitis on examination?

Answer 6

• May have no signs
• Jaundice
• Hepatosplenomegaly
• Spider naevi
• Palmar erythema
• Ascites

Question 7

what is the gold standard investigation for primary sclerosis cholangitis?•

Answer 7

1. MRCP and 2.ERCP
   o Will show stricturing and dilation of the biliary ducts.
   o Gives a ‘beaded appearance

Question 8

what is the first line investigation for primary sclerosis cholangitis?

Answer 8

USS

Question 9

what might the bloods show for primary sclerosis cholangitis?

Answer 9

o LFTs:
• High ALP + GGT
• Mildly elevated ALT + AST
• Low albumin + high bilirubin

Question 10

what antibodies might be seen in primary sclerosis cholangitis?

Answer 10

pANCA