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

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

PC

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

Investigations

Labs:

• LFT - raised bilirubin + ALP
• p-ANCA may be positive

Imaging:

• ERCP or MRCP are the standard diagnostic investigations, showing multiple biliary strictures giving a 'beaded' appearance
  • investigation of choice to visualise intra and extra hepatic bile ducts is MRCP

Special tests:

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