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 features

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