A familial case of primary biliary cirrhosis and autoimmune chronic active "lupoid" hepatitis.

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Primary biliary cirrhosis and autoimmune chronic active "lupoid" hepatitis were occurred in a 67-year-old woman and her 27-year-old daughter. The mother was asymptomatic but PBC was suspected by the increase of the serum alkaline phosphatase level. Positive anti-mitochondrial and anti-PDC antibody, and histological findings supported the diagnosis. Her daughter was suffered from acute hepatitis at 27 years old. Autoimmune chronic active "lupoid" hepatitis was diagnosed from the findings of hypergammaglobulinemia, positive anti-nuclear and anti-smooth muscle antibodies and good response to corticosteroid therapy. A common histocompatibility antigen was HLA-DR4.<BR>PBC and autoimmune chronic active hepatitis are both autoimmune liver disease in which liver damages occur by the immunological disorder. However, they have different target cells in immune response. Nevertheless, a similar genetic predisposition to immunologic abnormality is suggested in both PBC and autoimmune chronic active hepatitis in these cases.
社団法人日本肝臓学会の論文