Meroni P L, Tincani A, Sepp N, Raschi E, Testoni C, Corsini E, Cavazzana I, Pellegrini S, Salmaggi A
Allergy and Clinical Immunology Unit, Department of Internal Medicine, University of Milan, IRCCS Istituto Auxologico Italiano, Milan, Italy.
Lupus. 2003;12(12):919-28. doi: 10.1191/0961203303lu503oa.
Central nervous system (CNS) involvement in systemic lupus erythematosus (SLE) is common and results in different clinical manifestations. Several pathogenic mechanisms have been suggested to play a role in determining such a variety of clinical symptoms. The thrombophilic state associated to the presence of antiphospholipid antibodies has been suggested to be responsible for a noninflammatory vasculopathy which causes clear ischaemic events as well as alterations of the cerebral microcirculation that are likely associated to seizures, cognitive dysfunction or psychosis. Although less frequent, a true vasculitic process affecting cerebral circulation has also been reported. In both cases, brain endothelium does represent the target of the pathogenic mechanisms. Brain endothelial cells display peculiar functional and phenotypical characteristics in comparison with endothelial cells from other anatomical districts, raising the possibility that this might be the reason for its susceptibility in lupus disease. We review and present data suggesting that a higher and firmer expression of beta 2 glycoprotein I on endothelial cell membranes can be responsible for a selective damage/activation by circulating anti-beta 2 glycoprotein I, and that antiendothelial cell antibodies crossreact with brain endothelium and in some cases, specifically bind brain endothelial cells only in lupus patients with central nervous involvement.
中枢神经系统（CNS）受累于系统性红斑狼疮（SLE）很常见，并导致不同的临床表现。已提出几种致病机制在决定如此多样的临床症状中起作用。与抗磷脂抗体的存在相关的血栓形成倾向被认为是一种非炎症性血管病变的原因，这种病变会导致明显的缺血事件以及脑微循环改变，而这些改变可能与癫痫发作、认知功能障碍或精神病有关。尽管不太常见，但也有报道称存在影响脑循环的真正血管炎过程。在这两种情况下，脑内皮确实是致病机制的靶点。与来自其他解剖区域的内皮细胞相比，脑内皮细胞具有独特的功能和表型特征，这增加了这可能是其在狼疮疾病中易感性的原因的可能性。我们回顾并展示数据表明，内皮细胞膜上β2糖蛋白I的更高且更稳定的表达可能是循环抗β2糖蛋白I导致选择性损伤/激活的原因，并且抗内皮细胞抗体与脑内皮发生交叉反应，在某些情况下，仅在有中枢神经系统受累的狼疮患者中特异性结合脑内皮细胞。
