Youinou P, Isenberg D A, Kalsi J K, Dugoujon J M, Ravirajan C T, Muller S, Blanco F, Piette J C, Guillevin L, Jouquan J, Semana G, Salmon D, Shoenfeld Y, Bach J F
Brest University Medical School, France.
J Autoimmun. 1996 Dec;9(6):767-75. doi: 10.1006/jaut.1996.0099.
Our aim was to investigate links between systemic lupus erythematosus (SLE)-associated autoantibodies, idiotypes (Id) and genetic predisposition to their development. We studied four public Ids (16/6, WRI 176 beta, RT72 and RT84), identified the Km and Gm phenotypes and sought six selected autoantibodies in 32 SLE patients, 174 of their relatives and 15 spouses. Though anti-double-stranded DNA antibody was uncommon in the relatives (9%), the range of antinuclear reactivities was as broad in the relatives as in the probands. Antibodies to the synthetic peptide U1-RNP-A 35-38 were found in 56% of the patients, 28% of their relatives and 20% of the spouses, whereas antibodies to the Golgi apparatus was present in 7% of the patients, 26% of their relatives and 33% of the spouses. However, most of these family members were unaffected. RT84 Id was positively associated with antibodies to Sm-D peptide 1-20 and to Ro/SSA 60 kD peptide 304-324, but negatively associated with anti-dsDNA activity. The median of age was significantly lower in the RT84 Id-positive than in the RT84 Id-negative individuals. These data suggest that genetic as well as environmental factors are involved in the aetiology of SLE. In addition, RT84-carrying immunoglobulins (Ab2) might be directed to one of many cross-reactive Ids of dsDNA-binding antibodies (Ab1), perhaps down-regulating their production.
我们的目的是研究系统性红斑狼疮（SLE）相关自身抗体、独特型（Id）及其产生的遗传易感性之间的联系。我们研究了四种公共独特型（16/6、WRI 176β、RT72和RT84），确定了Km和Gm表型，并在32例SLE患者、174名其亲属和15名配偶中寻找六种选定的自身抗体。尽管抗双链DNA抗体在亲属中不常见（9%），但亲属中的抗核反应范围与先证者一样广泛。56%的患者、28%的亲属和20%的配偶中发现了针对合成肽U1-RNP-A 35-38的抗体，而7%的患者、26%的亲属和33%的配偶中存在针对高尔基体的抗体。然而，这些家庭成员中的大多数并未患病。RT84独特型与抗Sm-D肽1-20和抗Ro/SSA 60 kD肽304-324的抗体呈正相关，但与抗双链DNA活性呈负相关。RT84独特型阳性个体的年龄中位数显著低于RT84独特型阴性个体。这些数据表明，遗传因素以及环境因素都参与了SLE的病因。此外，携带RT84的免疫球蛋白（Ab2）可能针对双链DNA结合抗体（Ab1）的许多交叉反应独特型之一，可能下调其产生。
