Cellular immune response in primary biliary cirrhosis

The generation of immune responsiveness to self-antigen can result in the pathogenic autoimmune damage of tissues mediated by both humoral and cellular immune responses. Primary biliary cirrhosis (PBC) constitutes a model of autoimmune disease reflective of other organ-specific autoimmune feature. Although the etiology of PBC remained elusive, growing data suggest the role of cell-mediated immune response in the pathogenesis of PBC. Indeed, autoreactive CD4 as well as CD8 T cells have been characterized and their epitopes defined. Molecular mimicry is implicated in the initiation of these autoreactive T cell responses. Moreover, selective enrichment of NKT cells in the liver of PBC is demonstrated using CD1d tetramer.In this review, we shall focus on the recent advance of cell-mediated immune responses in PBC, which may be directly associated with inflammatory response in PBC.