Bruns Alessandra, Meyer Olivier
Service de rhumatologie, hôpital Bichat, APHP, 46, rue Henri-Huchard, 75018 Paris, France.
Joint Bone Spine. 2006 Dec;73(6):639-45. doi: 10.1016/j.jbspin.2006.05.006. Epub 2006 Sep 20.
Central nervous system (CNS) involvement in systemic lupus erythematosus (SLE) can produce a broad range of disease-specific neuropsychiatric manifestations that must be differentiated from infections, metabolic complications, and drug-induced toxicity. Despite the development of classification criteria by the American College of Rheumatology, the prevalence of neuropsychiatric systemic lupus erythematosus (NPSLE) varies widely across studies. Some of the neuropsychiatric manifestations are extremely rare, indicating a need for multicenter studies. Mechanisms that can lead to neuropsychiatric manifestations include intracranial vascular lesions (vasculitis and thrombosis); production of autoantibodies to neuronal antigens, ribosomes, and phospholipids; and inflammation related to local cytokine production. As a rule, no reference standard is available for establishing the diagnosis of NPSLE. Several investigations can be used to assist in the clinical diagnosis and to evaluate severity. Treatment remains largely empirical, given the absence of controlled studies. Variable combinations of corticosteroids, immunosuppressants, and symptomatic drugs are used according to the presumptive main pathogenic mechanism.
系统性红斑狼疮（SLE）累及中枢神经系统（CNS）可产生一系列特定疾病的神经精神表现，必须与感染、代谢并发症及药物所致毒性相鉴别。尽管美国风湿病学会制定了分类标准，但神经精神性系统性红斑狼疮（NPSLE）的患病率在各项研究中差异很大。部分神经精神表现极为罕见，这表明需要开展多中心研究。可导致神经精神表现的机制包括颅内血管病变（血管炎和血栓形成）；针对神经元抗原、核糖体和磷脂产生自身抗体；以及与局部细胞因子产生相关的炎症。通常，尚无用于确立NPSLE诊断的参考标准。多项检查可用于辅助临床诊断及评估病情严重程度。鉴于缺乏对照研究，治疗很大程度上仍基于经验。根据推测的主要致病机制，使用皮质类固醇、免疫抑制剂和对症药物的不同组合。
