Praprotnik S, Rozman B, Blank M, Shoenfeld Y
Department of Rheumatology, University Clinical Center, Ljubljana, Slovenia.
Wien Klin Wochenschr. 2000 Aug 25;112(15-16):660-4.
Antiendothelial cell antibodies (AECA), a heterogeneous group of antibodies quite distinct from the ANCA family, have been detected in variety of diseases which share a varying degree of vessel wall damage. This review is mainly focused on Wegener's granulomatosis, Takayasu's arteritis and Kawasaki syndrome, which provide the best examples to evaluate the pathogenic and prognostic value of AECA. There is increasing evidence to show that AECA might be pathogenic in inducing autoimmune vascular disease. It is relevant to note that the presence and titre of AECA has been correlated with disease activity in systemic vasculitis. Experimental in vitro and in vivo models support a potential pathogenic role for AECA in sustaining immune-mediated vessel inflammation. Rather than being cytotoxic to endothelial cells, AECA are able to up-regulate the expression of adhesion molecules (E-selectin, intercellular adhesion molecule-1 and vascular cell adhesion molecule-1) and to induce the secretion of cytokine and chemokine which, in turn, cause leukocyte recruitment and adhesion. A recent idiotypic animal model has provided further evidence that AECA can be pathogenic.
抗内皮细胞抗体（AECA）是一组与抗中性粒细胞胞浆抗体（ANCA）家族截然不同的异质性抗体，已在多种存在不同程度血管壁损伤的疾病中被检测到。本综述主要聚焦于韦格纳肉芽肿、大动脉炎和川崎综合征，它们为评估AECA的致病和预后价值提供了最佳实例。越来越多的证据表明，AECA可能在诱导自身免疫性血管疾病中具有致病性。值得注意的是，AECA的存在和滴度与系统性血管炎的疾病活动相关。体外和体内实验模型支持AECA在维持免疫介导的血管炎症中具有潜在致病作用。AECA并非对内皮细胞具有细胞毒性，而是能够上调黏附分子（E-选择素、细胞间黏附分子-1和血管细胞黏附分子-1）的表达，并诱导细胞因子和趋化因子的分泌，进而导致白细胞募集和黏附。最近的独特型动物模型提供了进一步证据，证明AECA具有致病性。
