Antimitochondrial antibody(AMA)-negative but antinuclear antibody(ANA)-positive primary biliary cirrhosis(PBC) developed in HLA-identical sisters.

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Two sisters had suffered for several years from chronic intrahepatic cholestasis of unknown origin, which clinically, histologically, and biochemically fulfilled the criteria of chronic nonsuppurative destructive cholangitis. AMA was not detected by standard immunofluorescent methods or immunoblotting on PDH, but ANA was positive at high titers in either serum. Their HLAs (class I, DR, DQ) were completely identical, but they lacked HLA DR8 which was previously reported to be closely related to PBC, especially in Kochi area. Immunosuppressive treatments with methylprednisolone and azathioprine for the elder sister was successful, suggesting present cases are compatible with "immunocholangitis", which was proposed as a clinical concept discriminating from PBC.
社団法人日本肝臓学会の論文