There have been significant advances in our understanding of the immunobiology of primary biliary cirrhosis (PBC) and, in particular, a rigorous dissection of not only the serologic abnormalities, including antimitochondrial autoantibodies (AMA), but also the definition of autoreactive CD4+ and CD8+ T cells [1]. Further, there is increasing evidence for the interplay of genetic and environmental factors in individual host susceptibility. One paradox has been the selected destruction of small bile ducts in PBC despite the presence of mitochondrial antigens in virtually all nucleated cells.
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Lleò et al. (2011) studied this question.
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