Provost T T, Talal N, Bias W, Harley J B, Reichlin M, Alexander E L
Department of Dermatology, Johns Hopkins Medical Institutions, Baltimore, MD 21205.
J Invest Dermatol. 1988 Oct;91(4):369-71. doi: 10.1111/1523-1747.ep12476204.
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.
Ro(SS-A)阳性的干燥综合征（SS）与红斑狼疮（LE）重叠的女性患者是一个临床和血清学上均一的群体，通常表现为显著的亚急性皮肤型红斑狼疮（SCLE）损害、皮肤血管炎、外周和中枢神经系统疾病、肺部疾病以及肾小球肾炎发生率较低。她们通常表现出类风湿因子、高球蛋白血症、抗核抗体以及Ro(SS-A)和La(SS-B)抗体活性。本研究表明，这些患者在免疫遗传学上也相似，HLA-B8、DR3、DRW6、DQ2和DRw52的频率在统计学上显著增加。这些SS/LE患者中有63%拥有扩展单倍型（P值6.0×10⁻³；相对风险9.5）HLA-B8、DR3、DQ2、DRw52。该SS/LE队列中100%为DR3或DRw6（P值小于或等于5.0×10⁻³；相对风险19.1）。这些患者中有50%是HLA DR3/DRw6杂合子（P值1.5×10⁻⁶；相对风险31.2）。因此，HLA-DR3和DRw6 Ro(SS-A)阳性的SS/LE患者可能拥有相似（即便不是独特）的与疾病易感性或免疫调节有关的DR区域DNA核苷酸序列。
