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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