Sjögren 症候群を合併した慢性肝内胆汁うっ滞症の3例

概要

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Three cases of chronic intrahepatic cholestasis with Sjögrens syndromes were reported. Sjögrens syndrome was diagnosed by clinical features, Schirmer test and sialography in case 1 and 2. In case 3, the syndrome was suspected by clinical pictures and positive Schirmer test. All cases were female of over middle age, and serum alkaline phosphatase was makedly increased. Although increased IgM and positive anti-mitochondrial antibody suggested primary biliary cirrhosis in case 1 and 2, degenerative and necrotic alteration of the bile ducts which was pathognomonic to primary biliary cirrhosis was not observed. In case 3, lymphocyte and plasma cell infiltration and decrease and destruction of bile ducts were evident histologically. Sjögrens syndrome is suggested to be a generalized disease with hypofunction of exocrine glands by autoimmune mechanism. In some cases of intrahepatic cholestasis including primary biliary cirrhosis, autoimmune reaction is suspected by the positive autoantibody. Interrelation of Sjögrens syndrome and intrahepatic cholestasis was discussed.