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Primary biliary cholangitis

From Surgopaedia

Previously called 'primary biliary cirrhosis'

Aetiology

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  • Unknown, but probably an environmental trigger in a genetically-susceptible person
  • Environmental
    • 25 per million in Australia; 250 per million in UK
    • Infections possibly causing molecular mimicry - viruses, bacteria
    • Chemical
  • Genetic
    • Clustering within families

Pathophysiology

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  • T-lymphocyte-mediated attack on small intralobular bile duct epithelial cells
  • Leads to gradual destruction and eventual disappearance, causing cholestasis
  • Eventual result is cirrhosis and liver failure
  • Anti-mitochondrial antibodies are the serological hallmark

Presentation

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  • Asymptomatic
  • Symptomatic
    • Fatigue
    • Pruritis
  • Associated autoimmune disorders

Diagnosis

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  • Suspect in patients with elevated ALP without anatomical obstruction; especially with previous autoimmune disease
  • Criteria
    • ALP >1.5x ULN
    • AMA at a titre of 1:40 or higher
    • Histological evidence of PBC

Management

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  • Liver protection
    • Immunise against hepatitis
    • Avoid alcohol
  • Medications
    • Ursodeoxycholic acid - slows progression, improves LFTs
  • Liver transplant can be considered
  • Look for and treat complications of cholestasis

Complications

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  • Cirrhosis
  • HCC
  • Metabolic bone disease

Prognosis

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  • Majority now have normal life expectancies
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