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

Abstract

Clinical studies and typing for HLA-A, HLA-B, HLA-C and HLA-DR loci on 13 family cases with familial Sjogren'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 Sjogren's syndrome. The patients with familial Sjogren's syndrome had same HLA antigens each other, but no HLA antigens specific for Sjogren'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 Sjogren's syndrome were not observed.From these results, it was concluded that the pathogenesis of Sjogren's syndrome is heterogeneous.Also we stressed that further collections of cases with familial Sjogren's syndrome of both clinical and subclinical type are necessary to make clear the factors which play a role on the development of Sjogren's syndrome.