Autoimmune disease of the liver which causes the destruction of the small bile ducts - liver only
Aetiology
Autoimmune
Much more common in women (10:1), especially middle-aged women
Pathophysiology
90% of people with PBC have anti-mitochondrial antibodies (AMAs) against pyruvate dehydrogenase complex (PDC-E2) - enzyme complex found in mitochondria
T cell mediated - CD4+ cells react against antibodies
Clinical features
Usually asymptomatic/incidental finding - most will not develop symptoms, and most will not develop cirrhosis/liver failure
Symptoms can include:
Fatigue
Itch without rash
Xanthelasma and xanthomas
Investigations
2 of:
Positive AMA
Cholestatic LFTs - ALP > ALT/AST, ↑ GGT, ↑ bilirubin
Liver biopsy
Management
Ursodeoxycholic acid
First line
Increases flow of bile to wash the toxic bile salts out