primary sclerosing cholangitis

Question 1

define primary sclerosing cholangitis

Answer 1

when the bile ducts inside and outside the liver become inflamed and scarred and eventually narrowed or blocked

Question 2

describe the pathology behind primary sclerosing cholangitis

Answer 2

1. biliary tree becomes strictured and fibrotic
2. obstruction to the flow of bile out of the liver
3. sclerosis- stiffening and hardening of the bile ducts
4. cholangitis- inflammation of the bile duct
5. eventually leads to liver inflammation, fibrosis and cirrhosis

Question 3

what are the risk factors for primary sclerosing cholangitis?

Answer 3

• male
• 30-40
• family history
• ulcerative colitis

Question 4

what are the symptoms for primary sclerosing cholangitis?

Answer 4

initially asymptomatic
-pruritus + fatigue
- jaundice
-charcots triad
-hepatosplenomegaly

Question 5

how Is primary sclerosing cholangitis diagnosed?

Answer 5

• LFTs
• serology
• U + E
• FBC

Question 6

what is the gold standard test for diagnosing primary sclerosing cholangitis?

Answer 6

MRCP
- may show bile duct lesions or strictures

Question 7

what would you find in serology if you had primary sclerosing cholangitis?

Answer 7

• pANCA
• ANA
• acL
• HBvsAg/ HCVAb -ve

Question 8

what is the treatment for primary sclerosing cholangitis?

Answer 8

• conservative symptom management
• cholestyramine for pruritus
• fat soluble for ADEK
• ERCP to dilate and stent any strictures

Question 9

PBC VS PSC

Answer 9

PBC
- women - AI
- associated w other autoimmune
-symptoms- pruritus , fatigue, jaundice
-diagnosis- LFT, serology ( AMA +ve), AUSS, biopsy
- treatment- ursdeoxycholic acid + cholestryamine

PSC
- Men - atypical
- associated with IBD
- Symptoms- pruritus, charcots triad, IBD signs
- diagnosis- LFT, serology ( panca), MRCP
- Treatment- conservative, cholestyramine