Primary Biliary Cirrhosis - Pathophysiology
Environmental Risk Factors: Geographic location, Smoking, Microbial triggers, Xenobiotics, Nail polish
Epigentics Risk Factors: miRNA profiles, Oestrogen (female-predominant disease), DNA methylation
Genetic Risk Factors: HLA variants, Non-HLA variants (immunoregulatory gene loci), Personal/family history of autoimmune disease, First-degree relative with PBC
Pathophysiology:
- Coordinated T & B cell mediated autoimmune cascade with T-cell mediated BEC injury
- Loss of the protective bicarbonate-rich umbrella around BECs and gradual loss of bile ducts
- Progressive inflammation and biliary fibrosis
- Cholestasis
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