Primary sclerosing cholangitis

Question 1

biliary disease of unknown aetiology characterised by […] and […] of intra and extra-[…] […] ducts.

Answer 1

biliary disease of unknown aetiology characterised by inflammation and fibrosis of intra and extra-hepatic bile ducts.

Question 2

Associations

[…] […]: 4% of patients with UC have PSC, 80% of patients with PSC have UC
[…] (much less common association than UC)
[…]

Answer 2

Associations

ulcerative colitis: 4% of patients with UC have PSC, 80% of patients with PSC have UC
Crohn’s (much less common association than UC)
HIV

Question 3

Features

[…]
[…], pruritus
raised […] + […]
[…][…][…] pain
fatigue

Answer 3

Features

cholestasis
jaundice, pruritus
raised bilirubin + ALP
right upper quadrant pain
fatigue

Question 4

Investigations

endoscopic retrograde cholangiopancreatography (ERCP) or magnetic resonance cholangiopancreatography (MRCP) are the standard diagnostic investigations, showing multiple biliary strictures giving a ‘[…]’ appearance - […][…][…][…]

[…]-[…] may be positive

Answer 4

endoscopic retrograde cholangiopancreatography (ERCP) or magnetic resonance cholangiopancreatography (MRCP) are the standard diagnostic investigations, showing multiple biliary strictures giving a ‘beaded’ appearance
p-ANCA may be positive

Question 5

Complication

Answer 5

cholangiocarcinoma

Question 6

Which antibody may be positive in PSC

Answer 6

p-ANCA

Question 7

What will ERCP show in PSC