Innate Immunity and Primary Biliary Cirrhosis: Activated Invariant Natural Killer T Cells Exacerbate Murine Autoimmune Cholangitis and Fibrosis §Δ

Our data suggests a primary role of innate immunity in the exacerbation of autoimmune cholangitis and also become a logical explanation for the recurrence of PBC following liver transplantation in the absence of major histocompatability complex compatibility. We submit that PBC begins with loss of tolerance to PDC-E2 and a multilineage antimitochondrial response in which autoreactive CD8(+) T cells are critical. However, the perpetuation of disease and its exacerbation will also be modulated by innate immune mechanisms.