Abstract: Background Neuropsychiatric systemic lupus erythematosus (NP-SLE) is the central nervous system (CNS) involvement of systemic lupus erythematosus (SLE). The diagnosis of NP-SLE may be difficult due to the lack of specific biomarker. CNS cerebrospinal fluid (CSF) cytology is diagnostic significant to CNS autoimmune disease. This paper described the characteristics of CSF cytology and evaluated its diagnostic value in NP-SLE. Methods Seventy-six eligible patients with clear diagnosis of NP-SLE were collected for CSF cytological examinations. Results The CSF cytology findings of 25 cases in 76 were abnormal, among which 16 cases showed lymphocytic inflammatory reactions; 8 cases had slight increase of neutrophile granulocyte percent; 9 cases showed lymphocyte-neutrophile inflammation. Activated lymphocytes together with monocytes were present in 24 cases. Among those cases, abnormal endocytosis of monocytes, which presented as monocytes phagocytosing lymphocytes or plasmocytes, was shown in 17 cases; plasmocytes were found in 17 cases. Conclusion The diagnosis of NP-SLE is based on clinical, neuroimaging and CSF studies. Among these methods, the CSF cytology findings are quite useful in practice, since the CSF cytological inflammatory reactions, especially the presentation of abnormal phagocytes in CSF is typical in NP-SLE and indicates its vasculitic mechanism.

Key words: Lupus vasculitis, central nervous system, Mental disorders , Cerebrospinal fluid, Cytological techniques

摘要： 研究背景 神经精神狼疮是系统性红斑狼疮累及中枢神经系统的严重并发症，缺乏特异性诊断方法。脑脊液细胞学检查对诊断中枢神经系统自身免疫性疾病具有重要价值，但目前尚缺乏这方面的研究报道，我们尝试对本组病例进行分析总结，以为临床提供一些参考。方法 对76例临床诊断明确且符合入组条件的神经精神狼疮患者进行脑脊液细胞学检查。结果 25例患者脑脊液细胞学检查异常，16例以淋巴细胞反应为主，其中8 例同时伴有中性粒细胞比例轻度升高、9 例为淋巴细胞? 中性粒细胞混合性炎症反应。24例淋巴细胞激活并伴单核细胞激活，其中17例可见单核细胞异常吞噬现象，表现为单核细胞吞噬淋巴细胞、浆细胞和（或）红细胞等；17例脑脊液中发现浆细胞。结论 神经精神狼疮的诊断基于临床、神经影像学和脑脊液检查等方法。其脑脊液细胞学呈现炎症反应和异常吞噬细胞，具有一定特点和诊断意义。

关键词: 狼疮血管炎, 中枢神经系统, 精神障碍, 脑脊髓液, 细胞学技术