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).

Aetiology:

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 fibrosis and cirrhosis.

The classic presentation is itching in a middle-aged woman

Primary biliary cholangitis - the M rule:

• IgM
• anti-Mitochondrial antibodies, M2 subtype
• Middle aged females

Associations

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

PC

• early: may be asymptomatic (e.g. raised ALP on routine LFTs) or fatigue, pruritus (main symptoms)
• cholestatic jaundice
• hyperpigmentation, especially over pressure points