Introduction

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

Epidemiology

  • Incidence: 2.00 cases per 100,000 person-years
  • Peak incidence: 40-50 years
  • Sex ratio: more common in males 2:1
Condition Relative
incidence
Primary biliary cholangitis6.00
Primary sclerosing cholangitis1
<1 1-5 6+ 16+ 30+ 40+ 50+ 60+ 70+ 80+

Aetiology

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

Clinical features

Features

Investigations

Investigation
  • endoscopic retrograde cholangiopancreatography (ERCP) or magnetic resonance cholangiopancreatography (MRCP) are the standard diagnostic investigations, showing multiple biliary strictures giving a 'beaded' appearance
  • ANCA may be positive
  • there is a limited role for liver biopsy, which may show fibrous, obliterative cholangitis often described as 'onion skin'

Complications

Complications


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