Primary Sclerosing Cholangitis

Chronic autoimmune condition that results in the fibrosis and destruction of intra-hepatic and extra-hepatic bile ducts

Aetiology

  • 80% have associated IBD (more likely to be UC)
  • Most commonly affects males, median age at diagnosis is 35 years
  • Genetic

Pathophysiology

  • Bile duct strictures impede the flow of bile, causing recurrent clinical cholangitis
  • Can lead to cirrhosis and liver failure

Clinical presentation

Symptoms

  • May be asymptomatic (presenting with abnormal LFTs or hepatomegaly)
  • RUQ pain
  • Fatigue, weight loss, fevers and sweats
  • Jaundice and pruritus

Signs

  • Jaundice
  • Hepatomegaly

Investigations

LFTs

  • Cholestatic - ALP > ALT/AST, ↑ GGT and bilirubin

Imaging

  • USS
  • MRCP - standard procedure to visualise the intrahepatic and extrahepatic bile ducts, multiple beaded biliary strictures will be seen
    • ERCP is an alternative but is invasive

Management

  • Maintain bile flow - balloon dilation, stent placement
  • Monitor for cholangiocarcinoma and colorectal cancer