Primary biliary cirrhosis (PBC) is supposed to be a disease entity in which autoimmune mechanisms may be operative, characterized by chronic nonsuppurative destructive cholangitis (CNSDC), cholestasis and epithelioid granuloma formation in its early stage. Thus far, although the pathogenesis of the disease remains largely unknown, there are a number of reports indicating hepatic lesions induced following graft-versus-host reaction (GVHR) in both human and experimental animals after the bone marrow transplantation. Recently, we demonstrated the bile duct lesions resembling CNSDC and the formation of epithelioid granuloma in the (B6xbm12) F1 mice with MHC class II-disparate GVHR, postinjection of spleen cells of parental B6 mice via the tail vein. By immunohistochemical examinations, both CD4^+ and CD8^+ T cells were found around the bile duct, and some of them were infiltrating among the bile duct epithelia on which the MHC class II antigen was aberrantly expressed. These findings indicate that MHC class II antigen might play an important role in the destruction of bile duct cells in PBC as well as in MHC class II-disparate GVHR.
2) 原発性胆汁性肝硬変 : 新しい動物モデルによる病態の解析(シンポジウム 自己免疫疾患, 第445回新潟医学会)
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