Primary sclerosis cholangitis

Question 1

What is primary sclerosing cholangitis?

Answer 1

Primary sclerosing cholangitis is a biliary disease of unknown aetiology characterised by inflammation and fibrosis of intra and extra-hepatic bile ducts.

Question 2

What can primary sclerosing cholangitis have?

Answer 2

positive p-ANCA

Question 3

Associations of primary sclerosing cholangitis?

Answer 3

• 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 4

Clinical presentation?

Answer 4

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

Question 5

Investigations?

Answer 5

• 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
• there is a limited role for liver biopsy, which may show fibrous, obliterative cholangitis often described as ‘onion skin’

Question 6

Complications?

Answer 6

• cholangiocarcinoma (in 10%)
• increased risk of colorectal cancer