Primary sclerosising cholangitis

Question 1

What is primary sclerotising cholangitis?

Answer 1

Biliary inflammation of unknown aetiology

Question 2

What is PSC charactersied by?

Answer 2

inflammation and fibrosis of intra and extra hepatic ducts

Question 3

What is the epid for PSC?

Answer 3

1. M>f

2. 20-40s

Question 4

What are the signs and symptoms of PSC?

Answer 4

1. Pruritus
2. Cholestatic jaundice
3. RUQ pain
4. Fatigue

Question 5

What is the diagnostic feature of PSC?

Answer 5

Diagnostic feature: Beaded appearance of bile ducts on MRCP

“Onion skin fibrosis”

Question 6

What are associastions with PSC?

Answer 6

UC

Question 7

What Ix are done for PSC?

Answer 7

1. Bloods
2. ERCP / MRCP – bead on a string appearance
3. Biopsy – “onion skin” fibrosis and obliteration of ducts

Question 8

What do bloods show in PSC?

Answer 8

pANCA +ve (not specific)

Question 9

What does ERCP/MRCP show in PSC?

Answer 9

bead on a string appearance - BEST ONE ERCP

Question 10

What does biopsy show in PSC?

Answer 10

“onion skin” fibrosis and obliteration of ducts

Question 11

What is the management for PSC?

Answer 11

Screening for risk of cholangiocarcinoma

Question 12

What is done at diagnosis to screen?

Answer 12

Ca 19-9 and USS / MRCP

Question 13

How often is the screening for PSC turning into cholagniocarcinoma?

Answer 13

1. F/U: LFTs and tumours markers checked every 6months. Imaging every 12 months
2. Proceed to ERCP and biopsies of any suspicious lesions

Question 14

What are possible complications of PSC?

Answer 14

Cirrhosis, cholangiocarcinoma