Ro(SS-A) positive Sjogren's/lupus erythematosus (SC/LE) overlap patients are associated with the HLA-DR3 and/or DRw6 phenotypes.
Ro(SS-A) positive female Sjögren's syndrome (SS) lupus erythematosus (LE) overlap patients are a clinically and serologically homogeneous group generally demonstrating prominent subacute cutaneous lupus erythematosus (SCLE) lesions, cutaneous vasculitis, peripheral and central nervous system disease, pulmonary disease, and a low frequency of glomerulonephritis. They commonly demonstrate rheumatoid factor, hypergammaglobulinemia, antinuclear and Ro(SS-A) La(SS-B) antibody activity. This study indicates that these patients are also immunogenetically similar, sharing a statistically significant increased frequency of HLA-B8, DR3, DRW6, DQ2, and DRw52. Sixty-three percent of these SS/LE patients possess the extended haplotype (P-value 6.0 X 10(-3); RR 9.5) HLA-B8, DR3, DQ2, DRw52. One hundred percent of this SS/LE cohort was DR3 or DRw6 (P-value less than or equal to 5.0 X 10(-3); relative risk 19.1). Fifty percent of these patients were HLA DR3/DRw6 heterozygotes (P-value 1.5 X 10(-6); relative risk 31.2). Thus, HLA-DR3 and DRw6 Ro(SS-A) positive SS/LE patients may possess a similar, if not unique, DR region DNA nucleotide sequence involved in disease susceptibility or immune regulation.
Provost
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TT
Department of Dermatology, Johns Hopkins Medical Institutions, Baltimore, MD 21205.
Talal
N
N
Bias
W
W
Harley
J B
JB
Reichlin
M
M
Alexander
E L
EL
D001324:Autoantigens / Q000032:analysis*; D005260:Female; D006684:HLA-DR Antigens / Q000235:genetics*; D015803:HLA-DR3 Antigen; D015808:HLA-DR6 Antigen; D006239:Haplotypes; D006579:Heterozygote; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000235:genetics / Q000276:immunology*; D010641:Phenotype; D020733:RNA, Small Cytoplasmic; D012261:Ribonucleoproteins; D012307:Risk Factors; D012859:Sjogren's Syndrome / Q000235:genetics / Q000276:immunology*; D000099207:SS-A Antigen
D001324:Autoantigens; D006684:HLA-DR Antigens; D015803:HLA-DR3 Antigen; D015808:HLA-DR6 Antigen; D020733:RNA, Small Cytoplasmic; D012261:Ribonucleoproteins; D000099207:SS-A Antigen; C116006:RO60 protein, human