|

Primary Biliary Cholangitis

Last updated:

Overview

Formerly known as primary biliary cirrhosis, primary biliary cholangitis (PBC) is an autoimmune liver disease characterised by the destruction of the small bile ducts of the liver, leading to cholestasis (the stopping of bile flow). Eventually, the damage leads to scarring, fibrosis, and eventually cirrhosis in its late stages. 

Epidemiology

  • The prevalence of PBC is around 35 per 100,000
  • Patients tend to present aged 40-60 years
  • PBC is 10 times more common in women
  • PBC is associated with other autoimmune diseases, particularly Sjögren’s syndrome

Risk Factors

  • Female sex
  • Personal or family history of autoimmune disease

Presentation

Patients tend to be asymptomatic or have very vague symptoms, typically itching and fatigue. Other features include:

  • Jaundice – this is not commonly seen in PBC
  • Raised ALP despite no symptoms
  • Hepatomegaly

Differential Diagnoses

Primary sclerosing cholangitis

  • PSC is more common in younger patients, particularly men
  • PSC is associated with ulcerative colitis

Investigations

  • LFTs:
    • Show cholestatic results, i.e. ALP and gamma-GT are more significantly increased compared to AST and ALT, which may be normal/slightly increased
  • Autoantibodies:
    • Elevated IgM
    • Anti-mitochondrial antibodies are present in up to 95% of patients
    • Anti-nuclear antibodies are present in around 30% of patients
  • Abdominal ultrasound:
    • Rules out obstruction
  • Magnetic resonance cholangiopancreatography (MRCP):
    • Rules out obstruction, which must be excluded

Management

Overview

  • 1st-line: ursodeoxycholic acid
    • This is a bile acid analogue whose mechanism is not fully understood. It both provides symptomatic relief and slows disease progression
    • It is thought to work by reducing hepatic secretion and synthesis leading to reduced cholesterol in gallstones allowing their dissolution
  • Cholestyramine is given for pruritus:
    • This is a bile acid sequestrant and keeps bile acids in the gut and prevents their reabsorption, so they are excreted
  • Liver transplants are performed in end-stage PBC

Complications

  • Liver fibrosis and cirrhosis
  • Hepatocellular carcinoma
  • Malabsorption of fats and fat-soluble vitamins
  • Hypercholesterolaemia
    • The use of statins is safe in patients with PBC

Author