Primary sclerosing cholangitis

Disease class: Cholangitis

Also known as PSC.


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

Features

• cholestasis: jaundice and pruritus
• right upper quadrant pain
• fatigue

Investigation

• ERCP is the standard diagnostic tool, 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

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

Typical history

A 40-year-old man with a history of ulcerative colitis presents with fatigue, jaundice and pruritus and right upper quadrant pain.