Immunopathology of primary biliary cirrhosis

Our understanding of the immunopathology of PBC has dramatically changed with the application of molecular biology techniques in clinical medicine. This has allowed, not only the possibility of characterizing mitochondrial autoantigens fully at the molecular level, but also the identification of specific sites on these molecules that are targetted by autoreactive B and T cells. In addition, the expression of cloned antigens has facilitated the development of the most reliable assays currently available for the detection of mitochondrial autoantibodies. The assessment of the pathogenic capacity of autoreactive T cells, as well as the characterization the PDC-E2 'look alike' molecule expressed on the cell membrane of PBC biliary epithelial cells, remain the major unsolved issues in this disease. Ideally, the continuous effort from both basic and clinical scientist in understanding the pathogenic mechanisms of PBC will lead to more specific, effective, and safer modalities of treatment.