Anti-Ro(SS-A) HLA-DR3-positive women: the interrelationship between some ANA negative, SS, SCLE, and NLE mothers and SS/LE overlap female patients.
During the past 15 years, the clinical spectrum associated with the anti-Ro(SS-A) antibody response has been defined. Various clinical presentations, including subacute cutaneous lupus erythematosus, the neonatal lupus syndrome, the Sjögren's syndrome/lupus erythematous overlap syndrome, and primary Sjögren's syndrome, have been detected in association with the anti-Ro(SS-A) response. The anti-Ro(SS-A) antibody response is associated with the HLA-DR2 and HLA-DR3 phenotypes. There is now a good deal of evidence to suggest that many anti-Ro(SS-A)-positive HLA-DR3 women are genetically closely related, sharing in common an enriched frequency of the HLA-DR3-linked B8, DQw2, and DRW52 phenotypes. DNA sequence studies have confirmed this genetic relationship. These studies have led us to the following conclusions. 1) The HLA-DR2 and HLA-DR3 associations with systemic lupus erythematosus and the HLA-DR3 association with Sjögren's syndrome are related to the anti-Ro(SS-A) antibody response and not to the clinical disease expression. 2) HLA-DR3 anti Ro-positive female patients with first-degree Sjögren's syndrome, subacute cutaneous lupus erythematosus, or Sjögren's syndrome, or who are asymptomatic, are immunogenetically closely related even though the clinical presentations are strikingly different. All these HLA-DR3 anti-Ro(SS-A) antibody-positive women are at risk to give birth to a child with the neonatal lupus syndrome.
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Department of Dermatology, John Hopkins School of Medicine, Baltimore, Maryland 21205-9977.
Watson
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D000328:Adult; D000974:Antibodies, Antinuclear / Q000032:analysis; D001324:Autoantigens / Q000032:analysis*; D005260:Female; D015803:HLA-DR3 Antigen / Q000032:analysis*; D006801:Humans; D007125:Immunogenetics; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000276:immunology*; D008178:Lupus Erythematosus, Cutaneous / Q000276:immunology*; D008180:Lupus Erythematosus, Systemic / Q000276:immunology*; D020733:RNA, Small Cytoplasmic; D012261:Ribonucleoproteins / Q000032:analysis*; D012859:Sjogren's Syndrome / Q000276:immunology*; D000099207:SS-A Antigen
D000974:Antibodies, Antinuclear; D001324:Autoantigens; D015803:HLA-DR3 Antigen; D020733:RNA, Small Cytoplasmic; D012261:Ribonucleoproteins; D000099207:SS-A Antigen; C116006:RO60 protein, human