van Dam A P
Central Laboratory of the Red Cross Blood Transfusion Service, Department of Autoimmune Diseases, Amsterdam, The Netherlands.
Rheumatol Int. 1991;11(1):1-11. doi: 10.1007/BF00290244.
The central nervous system (CNS) is clinically involved in approximately 40% of all systemic lupus erythematosis (SLE) patients. Minor psychiatric symptoms and abnormalities on neuropsychological testing are being detected with increasing frequency. This review summarizes current thinking concerning the diagnosis and pathogenesis of CNS lupus. The main symptoms of CNS lupus can be diffuse (generalized seizures, psychosis) or focal (stroke, peripheral neuropathies). Neuropsychiatric symptoms often occur in the first year of SLE, but are rarely the presenting symptoms of the disease. In studies on the pathology of CNS lupus, vasculopathy, infarcts and haemorrhages are often observed, whereas vasculitis is rare. Endocardial lesions and mural thrombi have also been reported in 33-50% of CNS lupus patients. In diagnostic imaging of the CNS, magnetic resonance imaging (MRI) scans often provide evidence for edema or small infarcts, both in focal and diffuse CNS lupus, whereas computerized tomography (CT) scans only show gross abnormalities. The first reports on position emission tomography (PET) scans in CNS lupus patients show decreased glucose uptake in the brain. The cerebral blood flow decreases during active diffuse and focal CNS lupus. The blood-brain barrier is somewhat more frequently impaired in diffuse CNS lupus. Intrathecal IgG and IgM production is observed in 25-66% of all CNS lupus patient. Various specificities of autoantibodies have been observed in CNS lupus. Of these, anticardiolipin (ACA) antibodies show a well-documented association with focal involvement of the CNS in SLE. These antibodies could cause thrombosis by interfering with the protein C pathway of fibrinolysis. In addition, they are associated with endocardial and valvular heart disease, which is often observed in SLE and which could cause embolism. The relation between ACA and diffuse CNS lupus is not yet clear. Low-avidity anti-DNA antibodies are also found in CNS lupus, possibly because of their cross-reaction with cardiolipin. Antineuronal antibodies and lymphocytotoxic antibodies have been associated with diffuse CNS lupus and abnormalities on neuropsychological testing. However, the population of these antibodies is rather heterogeneous and it has not been possible to assess a common target antigen. Therefore, it is still obscure whether there is also a second immune-mediated mechanism responsible for the development of the diffuse form of CNS lupus.
中枢神经系统（CNS）在所有系统性红斑狼疮（SLE）患者中约有40%会出现临床受累。神经心理学测试中轻微的精神症状和异常被检测到的频率越来越高。这篇综述总结了当前关于中枢神经系统狼疮的诊断和发病机制的观点。中枢神经系统狼疮的主要症状可以是弥漫性的（全身性癫痫发作、精神病）或局灶性的（中风、周围神经病变）。神经精神症状通常在SLE的第一年出现，但很少是该疾病的首发症状。在中枢神经系统狼疮的病理学研究中，经常观察到血管病变、梗死和出血，而血管炎则很少见。在33%至50%的中枢神经系统狼疮患者中也报告了心内膜病变和壁血栓。在中枢神经系统的诊断成像中，磁共振成像（MRI）扫描通常能为局灶性和弥漫性中枢神经系统狼疮的水肿或小梗死提供证据，而计算机断层扫描（CT）扫描仅显示明显异常。关于中枢神经系统狼疮患者正电子发射断层扫描（PET）扫描的首次报告显示大脑葡萄糖摄取减少。在活动性弥漫性和局灶性中枢神经系统狼疮期间，脑血流量会减少。在弥漫性中枢神经系统狼疮中，血脑屏障受损的情况更为常见。在所有中枢神经系统狼疮患者中，25%至66%观察到鞘内IgG和IgM产生。在中枢神经系统狼疮中观察到了各种自身抗体的特异性。其中，抗心磷脂（ACA）抗体与SLE中枢神经系统的局灶性受累有充分记录的关联。这些抗体可能通过干扰纤维蛋白溶解的蛋白C途径导致血栓形成。此外，它们与心内膜和瓣膜性心脏病有关，这在SLE中经常观察到，并且可能导致栓塞。ACA与弥漫性中枢神经系统狼疮之间的关系尚不清楚。在中枢神经系统狼疮中也发现了低亲和力抗DNA抗体，可能是因为它们与心磷脂发生交叉反应。抗神经元抗体和淋巴细胞毒性抗体与弥漫性中枢神经系统狼疮以及神经心理学测试中的异常有关。然而，这些抗体的群体相当异质，并且无法评估共同的靶抗原。因此，对于弥漫性中枢神经系统狼疮的发生是否还存在第二种免疫介导机制仍然不清楚。
