What is Primary sclerosing cholangitis?

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

What are the main clinical features of Primary sclerosing cholangitis?

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

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

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

What are the common complications of Primary sclerosing cholangitis?

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