Association of DR3 with susceptibility to and severity of primary Sjögren's syndrome in a family study.
A study of HLA and primary Sjögren's syndrome (1 degree SS) was performed in 40 index cases and 180 relatives all of whom were Caucasian. The association of DR3 and 1 degree SS was confirmed. In probands, DR3 associated with extraglandular manifestations of 1 degree SS and homozygosity for DR3 associated with younger onset of disease. Familial clustering of 1 degree SS was evident. Definite or probable 1 degree 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 anti-nuclear factor, rheumatoid factor or Ro and DR3. We identified a group of young females expressing some criteria for 1 degree SS and the same immunogenetic markers. They may be at risk of full expression of 1 degree SS as they become older. Milder forms of 1 degree SS were common in older relatives but not DR3 associated. 1 degree SS in males was rare and mild irrespective of immunogenetic status. Symptoms of 1 degree SS in relatives were mild or absent. Such individuals will only be identified through a family study or a community survey.
Foster
H
H
Department of Rheumatology, Royal Victoria Infirmary, Newcastle upon Tyne.
Walker
D
D
Charles
P
P
Kelly
C
C
Cavanagh
G
G
Griffiths
I
I
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001324:Autoantigens / Q000032:analysis / Q000235:genetics; D004198:Disease Susceptibility; D005260:Female; D005819:Genetic Markers / Q000276:immunology; D015803:HLA-DR3 Antigen / Q000032:analysis* / Q000235:genetics; D006720:Homozygote; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D020733:RNA, Small Cytoplasmic; D012261:Ribonucleoproteins; D012307:Risk Factors; D012720:Severity of Illness Index; D012859:Sjogren's Syndrome / Q000453:epidemiology / Q000235:genetics / Q000276:immunology*; D000097585:SS-B Antigen; D000099207:SS-A Antigen
D001324:Autoantigens; D005819:Genetic Markers; D015803:HLA-DR3 Antigen; D020733:RNA, Small Cytoplasmic; D012261:Ribonucleoproteins; D000097585:SS-B Antigen; D000099207:SS-A Antigen; C116006:RO60 protein, human