Division of Rheumatology, Department of Medicine and Department of Pathology, Capital Health and Dalhousie University, Halifax, NS B3H 4K4, Canada.
Nat Rev Rheumatol. 2014 Jun;10(6):338-47. doi: 10.1038/nrrheum.2014.15. Epub 2014 Feb 11.
Nervous system involvement in systemic lupus erythematosus (SLE) can manifest as a range of neurological and psychiatric features, which are classified using the ACR case definitions for 19 neuropsychiatric syndromes. Approximately one-third of all neuropsychiatric syndromes in patients with SLE are primary manifestations of SLE-related autoimmunity, with seizure disorders, cerebrovascular disease, acute confusional state and neuropathy being the most common. Such primary neuropsychiatric SLE (NPSLE) events are a consequence either of microvasculopathy and thrombosis, or of autoantibodies and inflammatory mediators. Diagnosis of NPSLE requires the exclusion of other causes, and clinical assessment directs the selection of appropriate investigations. These investigations include measurement of autoantibodies, analysis of cerebrospinal fluid, electrophysiological studies, neuropsychological assessment and neuroimaging to evaluate brain structure and function. Treatment involves the management of comorbidities contributing to the neuropsychiatric event, use of symptomatic therapies, and more specific interventions with either anticoagulation or immunosuppressive agents, depending upon the primary immunopathogenetic mechanism. Although the prognosis is variable, studies suggest a more favourable outcome for primary NPSLE manifestations compared with neuropsychiatric events attributable to non-SLE causes.
神经系统受累在系统性红斑狼疮（SLE）中可表现为一系列神经系统和精神症状，可根据 ACR 关于 19 种神经精神综合征的病例定义进行分类。大约三分之一的 SLE 患者的神经精神综合征为 SLE 相关自身免疫的原发性表现，最常见的是癫痫、脑血管病、急性意识混乱状态和神经病。此类原发性神经精神性 SLE（NPSLE）事件是微血管病和血栓形成或自身抗体和炎症介质的结果。NPSLE 的诊断需要排除其他原因，临床评估指导选择适当的检查。这些检查包括自身抗体的测量、脑脊液分析、电生理学研究、神经心理学评估和神经影像学评估以评估脑结构和功能。治疗包括管理导致神经精神事件的合并症、使用对症治疗，以及根据主要免疫发病机制进行更具体的抗凝或免疫抑制治疗。尽管预后各不相同，但研究表明原发性 NPSLE 表现的预后优于非 SLE 原因引起的神经精神事件。
