Immune dysfuction

Primary biliary cirrhosis (PBC) is considered as an autoimmune disease.

• it has a sex distribution similar to other autoimmune diseases
• most PBC patients have other autoimmune diseases
• loss of immune tolerance to self-mitochondrial proteins (1)

Evidence in support of autoimmunity includes:

• specific serum autoantibodies
• autoreactive T cells
• adaptive transfer of cholangitis using CD8+ T cells (in murine models)
• functional T regulatory defects
• female predominance
• autoimmune comorbidity
• MHC association -however, neither humoral nor cytotoxic destruction of the biliary epithelium has been demonstrated

Evidence against autoimmunity includes:

• absence of disease after autoantibody transfer (in mice)
• absence of correlation between titre of antimitochondrial antibodies and disease severity48
• failure to respond to immunosuppressive agents (based on limited data) (1).

Reference;

• (1) Selmi C et al. Primary biliary cirrhosis. Lancet. 2011 May 7;377(9777):1600-9.