Abstract: Neuropsychiatric systemic lupus erythematosus （NPSLE）is systemic lupus erythematosus （SLE） with central or peripheral nervous system involvement.NPSLE is the second leading cause of morbidity and mortality in patients with SLE， following lupus nephritis.The pathogenesis of NPSLE is complex， including the formation of autoantibodies， blood-brain barrier injury， thromboembolism， and inflammatory mediators.The most common symptom of NPSLE in children is headache. Other common symptoms include seizures， cognitive dysfunction， mood disorders， psychosis， and cerebrovascular disease.Currently， there is no unified diagnostic criteria for NPSLE， which requires comprehensive evaluation of clinical symptoms， serological tests， cerebrospinal fluid examination， imaging and other aspects， and the exclusion of other causes.Symptomatic therapy， glucocorticoid， immunosuppressant， and anticoagulant therapy are the main therapeutic methods for children with NPSLE.

Key words: systemic lupus erythematosus, neuropsychiatric systemic lupus erythematosus, child, diagnosis and treatment

摘要： 神经精神性狼疮（neuropsychiatric systemic lupus erythematosus， NPSLE）是系统性红斑狼疮（systemic lupus erythematosus，SLE）出现中枢或外周神经系统受累时的表现。NPSLE是SLE患者发病和死亡的第二大原因，仅次于狼疮性肾炎。NPSLE的发病机制错综复杂，自身抗体的形成、血脑屏障损伤、血栓栓塞、炎症介质等多种因素参与了发病过程。儿童NPSLE最常见的症状是头痛，其他常见症状有癫痫发作、认知功能障碍、情绪障碍、精神病和脑血管疾病等。NPSLE目前尚无统一的诊断标准，需要综合临床症状、血清学检测、脑脊液检查、影像学等多方面评估，并排除其他病因。对症治疗、糖皮质激素、免疫抑制剂、抗凝治疗是NPSLE患儿目前主要的治疗方法。

关键词: 系统性红斑狼疮, 神经精神性狼疮, 儿童, 诊治