ASSOCIATION OF DR3 WITH SUSCEPTIBILITY TO AND SEVERITY OF PRIMARY SJÖGREN'SYNDROME IN A FAMILY STUDY

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Abstract
A study of HLA and primary Sjögren's syndrome (1°SS) was performed in 40 index cases and 180 relatives all of whom were Caucasian. The association of DR3 and 1°SS was confirmed. In probands, DR3 associated with extraglandular manifestations of 1°SS and homozygosity for DR3 associated with younger onset of disease. Familial clustering of 1°SS was evident. Definite or probable 1°SS (Fox criteria) occurred with a prevalence of 4.4% in the relatives, exclusively in older female first degree relatives and was associated with DR3. The relative risk was greatest in those who expressed antinuclear factor, rheumatoid factor or Ro and DR3. We identified a group of young females expressing some criteria for 1°SS and the same immunogenetic markers. They may be at risk of full expression of 1°SS as they become older. Milder forms of 1°SS were common in older relatives but not DR3 associated. 1°SS in males was rare and mild irrespective of immunogenetic status. Symptoms of 1°SS in relatives were mild or absent. Such individuals will only be identified through a family study or a community survey

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