Introduction


Primary biliary cholangitis (previously referred to as primary biliary cirrhosis) is a chronic liver disorder typically seen in middle-aged females (female:male ratio of 9:1). The aetiology is not fully understood although it is thought to be an autoimmune condition. Interlobular bile ducts become damaged by a chronic inflammatory process causing progressive cholestasis, which may eventually progress to cirrhosis. The classic presentation is itching in a middle-aged woman.

Epidemiology

  • Incidence: 12.00 cases per 100,000 person-years
  • Peak incidence: 50-60 years
  • Sex ratio: more common in females 8:1
Condition Relative
incidence
Primary biliary cholangitis1
Primary sclerosing cholangitis0.17
<1 1-5 6+ 16+ 30+ 40+ 50+ 60+ 70+ 80+

Aetiology

Associations
  • Sjogren's syndrome (seen in up to 80% of patients)
  • Rheumatoid arthritis
  • Systemic sclerosis
  • Thyroid disease

Clinical features

  • Early: may be asymptomatic (e.g. raised ALP on routine LFTs) or fatigue, pruritus
  • Cholestatic jaundice
  • Hyperpigmentation, especially over pressure points
  • Around 10% of patients have right upper quadrant pain
  • Xanthelasmas, xanthomata
  • Also: clubbing, hepatosplenomegaly
  • Late: may progress to liver failure

Investigations

  • Anti-mitochondrial antibodies (AMA) M2 subtype are present in 98% of patients and are highly specific
  • Smooth muscle antibodies in 30% of patients
  • Raised serum IgM

Management

  • Pruritus: cholestyramine
  • Fat-soluble vitamin supplementation
  • Ursodeoxycholic acid
  • Liver transplantation e.g. if bilirubin > 100 (PBC is a major indication) - recurrence in graft can occur but is not usually a problem

Complications