<aside> 💡 autoimmune, slowly progressive, cholestatic, liver disease targeting small & medium sized intrahepatic bile ducts

• characterized by a triad of chronic cholestasis, circulating anti-mitochondrial antibodies (AMA), and liver biopsy findings </aside>

Demographics

• 40-50y females 90%
• geographic clustering England, Scotland & northern USA.

Aetiology

• ? Environmental + genetic (with other AID’s)
• associated with other autoimmune disorders & 70% Sjogren’s (drynes of eyes, salivary destruction)

Risk Factors

• Family history
• Clustering of autoimmunity
• Smoking
• History of UTI

Pathophysiology

• Immune-mediated biliary epithelial injury with subsequent cholestasis, ductopenia and progressive biliary fibrosis