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 CD 1d 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. (C) 2003 Elsevier B.V. All rights reserved.