Department of Pathophysiology, School of Medicine, University of Athens, 75 M Asias st, 11527 Athens, Greece.
J Autoimmun. 2010 Nov;35(3):256-64. doi: 10.1016/j.jaut.2010.06.016.
A common serologic finding in systemic autoimmune diseases is the presence of autoantibodies against intracellular autoantigens. Although their pathogenesis is not fully understood, autoantibodies are important tools for establishing diagnosis, classification and prognosis of autoimmune diseases. In Systemic Lupus Erythematosus (SLE) and Sjögren's syndrome (SS) autoantibodies mainly target multicomponent ribonucleoprotein complex Ro/La RNP. The last years, the main characteristics, the clinical significance of the anti-Ro/SSA and anti-La/SSB autoantibodies, their biologic function, as well as their B-cell antigenic determinants (epitopes) have been addressed. More specifically, the structural characteristics and clinical associations of epitopes along with their utility as tools to investigate the autoimmune response have been investigated in detail. New insights for the pathogenetic role of epitopes in initiation, propagation and regulation of systemic autoimmunity have been emerged. In this regard, the role of epitope spreading in the diversification of autoimmune response and the anti-idiotypic antibodies in the regulation of autoantibodies (idiotypic) response are addressed.
在系统性自身免疫性疾病中,常见的血清学发现是存在针对细胞内自身抗原的自身抗体。尽管其发病机制尚未完全阐明,但自身抗体是建立自身免疫性疾病诊断、分类和预后的重要工具。在红斑狼疮(SLE)和干燥综合征(SS)中,自身抗体主要针对多成分核糖核蛋白复合物 Ro/La RNP。近年来,抗 Ro/SSA 和抗 La/SSB 自身抗体的主要特征、临床意义、生物学功能以及 B 细胞抗原决定簇(表位)已得到解决。更具体地说,已经详细研究了表位的结构特征和临床关联及其作为研究自身免疫反应的工具的用途。在启动、传播和调节系统性自身免疫方面,表位的致病作用的新见解已经出现。在这方面,探讨了表位扩展在自身免疫反应多样化中的作用,以及抗独特型抗体在调节自身抗体(独特型)反应中的作用。