immune dysfuction
Last reviewed 08/2021
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).
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