Primary Biliary Cholangitis

Presentation

  • Itch (common)
  • Fatigue (common)
  • Dry eyes and dry mouth (common)
  • Sleep disturbance (common)
  • Postural dizziness/blackouts

History

  • Female
  • 45-60 years old
  • Past medical history of autoimmune disease (common)
  • Family history of autoimmune disease (common)
  • History of hypercholesterolaemia (common)
  • Drug history

Differentials

  • Bile duct obstruction - However pain much more prominent in PBC. PBC differentiated by presence of AMA
  • Primary sclerosing cholangitis - PSC is associated with younger males and inflammatory bowel disease
  • Drug induced cholestasis

Examination

  • Hepatomegaly
  • Signs of chronic liver disease (uncommon)
    • Jaundice
    • Ascites
    • Splenomegaly

Laboratory investigations

LFTs

  • Raised ALP
  • Rasied ALT
  • Raised bilirubin
  • Low serum albumin
  • Antimitochondrial antibodies (AMA) present

Management

++++Aims:

  • Slow or stop progression of the disease to prevent the development of cirrhosis
  • Manage symptoms to improve quality of life

Managing disease progression

  • First-line treatment is ursodeoxycholic acid
  • Liver transplant for end-stage disease

Managing symptoms

  • Colestyramine (taken at least 4 hours after ursodeoxycholic acid to prevent the two agents binding)
  • No current treatment for fatigue

Complications

  • Hypercholesterolaemia
  • Osteoporosis
  • Portal hypertension secondary to cirrhosis

Prognosis

  • Mortality significantly increased in PBC patients due to both liver related and non-liver related causes of deaths
  • However, given the age of presentation with the disease, many patients die of other causes before reaching end-stage liver disease
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