Primary biliary cholangitis

Primary biliary cholangitis

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)

Primary biliary cholangitis - the M rule - IgM - anti-Mitochondrial antibodies, M2 subtype - Middle aged females

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Importance: 73

. 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

Associations

• Sjogren's syndrome (seen in up to 80% of patients)
  Sjogren's syndrome is common in patients with PBC
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  Importance: 49
• 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

Diagnosis

• immunology
  • anti-mitochondrial antibodies (AMA) M2 subtype
    Primary biliary cholangitis - the M rule - IgM - anti-Mitochondrial antibodies, M2 subtype - Middle aged females
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    Importance: 73
    are present in 98% of patients and are highly specific
  • smooth muscle antibodies in 30% of patients
  • raised serum IgM
    Primary biliary cholangitis - the M rule - IgM - anti-Mitochondrial antibodies, M2 subtype - Middle aged females
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    Importance: 73
• imaging
  Imaging is required before making a diagnosis of PBC to exclude extrahepatic biliary obstruction
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  Importance: 74
  • required before diagnosis to exclude an extrahepatic biliary obstruction (typically a right upper quadrant ultrasound or magnetic resonance cholangiopancreatography (MRCP)

Management

• first-line: ursodeoxycholic acid
  Ursodeoxycholic acid is the first-line medication for primary biliary cholangitis to slow disease progression
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  Importance: 55
  • slows disease progression and improves symptoms
• pruritus: cholestyramine
  Cholestyramine is used to provide symptomatic relief of pruritus in PBC
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  Importance: 56
• fat-soluble vitamin supplementation
• liver transplantation
  • e.g. if bilirubin > 100 (PBC is a major indication)
  • recurrence in graft can occur but is not usually a problem

Complications

• cirrhosis → portal hypertension → ascites, variceal haemorrhage
• osteomalacia
  osteomalacia
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  and osteoporosis
• significantly increased risk of hepatocellular carcinoma (20-fold increased risk)
  significantly increased risk of hepatocellular carcinoma (20-fold increased risk)
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+PassMedicine Notes

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

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

Diagnosis

• immunology
• 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
• imaging
• required before diagnosis to exclude an extrahepatic biliary obstruction (typically a right upper quadrant ultrasound or magnetic resonance cholangiopancreatography (MRCP)

Management

• first-line: ursodeoxycholic acid
• slows disease progression and improves symptoms
• pruritus: cholestyramine
• fat-soluble vitamin supplementation
• liver transplantation
• e.g. if bilirubin > 100 (PBC is a major indication)
• recurrence in graft can occur but is not usually a problem

Complications

• cirrhosis --> portal hypertension --> ascites, variceal haemorrhage
• osteomalacia and osteoporosis
• significantly increased risk of hepatocellular carcinoma (20-fold increased risk)