Primary sclerosing Cholangitis

Question 1

Define Primary sclerosing Cholangitis

Answer 1

Cholestatic liver disease (less prod of bile or blockage of bile out)
Chronic progressive inflammatory disease-inflam and fibrosis of intra and extra hepatic ducts->multifocal stricture formation

Difference from secondary SC is no real cause (trauma, pancreatitis, ischemia etc)

either large duct PSC-more common
Small duct PSC-only visible via microscopy

Question 2

Aetiology and risk factors of Primary sclerosing Cholangitis

Answer 2

Not well understood
Possibly autoimmune (Auto Ab’s, and often found with other AID)
Inflam causes fibrosis-stricture of ducts as remodel-and bile block causes stones (and contribute to damage)
Blockage contributes to jaundice, pruritus, bacterial cholangitis and progression to billiary cirrhosis
Continued remodelling can progress to cancer, end stage liver disease

Risk factors:
Northern europe
Hx of IBD-Ulcerative colitis
Male sex
FHx
age 40-50

Question 3

Epidiemology of Primary sclerosing Cholangitis

Answer 3

rare-3.6 per 100000
more common in Northern Europe and US
Large duct 80% of cases
2/3 have UC with

Question 4

Signs and Sx of Primary sclerosing Cholangitis

Answer 4

Nothing to specific
RUQ pain (30%)
Jaundice and pruritus (obstructive jaundice)

weight loss (no bile)
Fever (bacterial cholangitis)

even less specific/common:
Stratthorea,

after BAD liver involvement
Ascites, splenomegaly, encelopathy

Question 5

Investigations of Primary sclerosing Cholangitis

Answer 5

LFT’s-obstructive jaundice (ALP high, GGT and bilirubin raised, ALT fine-ish)

No specific auto-antibodies but some are there

ERCP/MRCP-see block in large duct PSC
take sample in ERCP for small duct PSC