Primary Sclerosing Cholangitis

Question 1

what is primary sclerosing cholangitis?

Answer 1

biliary disease of unknown aetiology

Question 2

what is primary sclerosing cholangitis characterised by?

Answer 2

inflammation and fibrosis of intra- and extra-hepatic bile ducts

Question 3

what is primary sclerosing cholangitis associated with?

Answer 3

• ulcerative colitis - 80% of patients with PSC have UC
• crohns
• HIV

Question 4

what are the features of primary sclerosing cholangitis?

Answer 4

• cholestasis
• right upper quandrant pain
• fatigue

Question 5

how does cholestasis present?

Answer 5

• jaundice
• pruritus
• raised bilirubin + ALP

Question 6

what is cholestasis?

Answer 6

reduced or stopped bile flow

Question 7

what is the best investigation of choice in primary sclerosing cholangitis?

Answer 7

magnetic resonance cholangiopancreatography (MRCP)

Question 8

what does MRCP show in primary sclerosing cholangitis?

Answer 8

multiple biliary strictures giving a ‘beaded’ appearance

Question 9

what antibody can be positive in primary sclerosing cholangitis?

Answer 9

p-ANCA

Question 10

what complications can occur secondary to primary sclerosing cholangitis?

Answer 10

• cholangiocarcinoma
• increased risk of colorectal cancer