All::Gastroenterology::Diseases::Primary sclerosing cholangitis

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What is Primary sclerosing cholangitis?

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

Primary sclerosing cholangitis

What are the main clinical features of Primary sclerosing cholangitis?

  • cholestasis -> jaundice, pruritus
  • right upper quadrant pain
  • fatigue

Primary sclerosing cholangitis

How is Primary sclerosing cholangitis diagnosed?

  • ERCP/MRCP are the standard diagnostic investigations, showing multiple biliary strictures giving a 'beaded' appearance
  • p-ANCA may be positive

Primary sclerosing cholangitis

What are the main associations of Primary sclerosing cholangitis?

  • 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

Primary sclerosing cholangitis

What are the common complications of Primary sclerosing cholangitis?

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

Primary sclerosing cholangitis