Familial Sjögren's syndrome Reports of three family cases and a review of 13 family cases reported in Japan

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Clinical studies and typing for HLA-A, HLA-B, HLA-C and HLA-DR loci on 13 family cases with familial Sjögren's syndrome in Japan including three family cases which have experienced in our institute were performed.In our cases, all patients had sicca syndrome alone without other connective tissue diseases. Four of the six patients had multiple involvements of organs such as renal tubular acidosis, chronic thyroiditis and partial hypopituitarism. Five of the six patients were subclinical Sjögren's syndrome. The patients with familial Sjögren's syndrome had same HLA antigens each other, but no HLA antigens specific for Sjögren's syndrome were found out in all patients.Also, in 13 family cases reported in Japan including the present cases, common HLA antigens specific for Sjögren's syndrome were not observed.From these results, it was concluded that the pathogenesis of Sjögren's syndrome is heterogeneous.Also we stressed that further collections of cases with familial Sjögren's syndrome of both clinical and subclinical type are necessary to make clear the factors which play a role on the development of Sjögren's syndrome.
日本臨床免疫学会の論文