| Characteristic | Characteristic seen in PBC? | Comment |
| Autoreactive immune responses to defined autoantigens | Yes | PDC association is among the strongest seen in any autoimmune disease |
| Female predisposition | Yes | 10 : 1 Female to male ratio although classical disease occurs in men ruling out an obligate female association |
| HLA association | Yes | Significant associations but with atypical alleles and weaker than in other autoimmune diseases |
| Induction of disease by sensitisation with autoantigen | Possibly | Observation awaits replication |
| Response to immunosuppressive therapy | Possibly | Comprehensive trials equivocal. Probably effective in selected individuals with significant inflammatory component |
| Production of disease by adoptive transfer of autoreactive cells and/or antibody | Possibly | Preliminary data suggestive. As yet unconfirmed |
| Spontaneous animal model with identical antigen specificity | No | No confirmed spontaneous model |
| Both children and adults affected | No | Disease in children is anecdotal |
| Autoantigen distribution matches disease distribution | No | PDC is universal but disease is highly restricted in its distribution |
HLA, human leukocyte antigen; PBC, primary biliary cirrhosis; PDC, pyruvate dehydrogenase complex.