What is the common presentation of Primary biliary cholangitis?

The classic presentation is itching in a middle-aged woman

What patient groups is Primary biliary cholangitis most common?

middle-aged females (female:male ratio of 9:1)

What are the main clinical features of Primary biliary cholangitis?

• 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

How is Primary biliary cholangitis diagnosed?

• 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 (USS/MRCP)

What is the management of Primary biliary cholangitis?

• 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

What are the main causes of Primary biliary cholangitis?

thought to be an autoimmune condition

What is the pathophysiology of Primary biliary cholangitis?

Interlobular bile ducts become damaged by a chronic inflammatory process causing progressive cholestasis which may eventually progress to cirrhosis

What are the main associations of Primary biliary cholangitis?

• Sjogren's syndrome (seen in up to 80% of patients)
• rheumatoid arthritis
• systemic sclerosis
• thyroid disease

What are the common complications of Primary biliary cholangitis?

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