摘要： 研究背景 神经精神狼疮临床表现多样，病死率高，诊断与鉴别诊断存在困难。本文探讨累及中枢神经系统的神经精神狼疮患者的临床特点及其与颅内感染性疾病的鉴别诊断。方法 回顾分析23例累及中枢神经系统的神经精神狼疮患者的临床表现、多项血清学和脑脊液检查结果、影像学和脑电图等临床资料。结果 临床表现为弥漫型神经精神症状者9 例、局灶型者14例。血清免疫球蛋白抗核抗体、抗双链DNA抗体、抗Sm抗体、抗核糖体P蛋白抗体、抗SSA 抗体和抗SSB 抗体阳性检出率分别为21/22、7/22 、1/14 、2/14 、9/14 和3/14 例，血清补体C3、C4降低者分别为14/20 和5/20 例；脑脊液白细胞计数和蛋白定量升高者为5/12 和7/12 例，葡萄糖和氯化物水平降低者为5/12 和6/12 例。影像学和部分患者（6 例）脑电图检查表现异常。结论 血清学、脑脊液影像学和脑电图检查均可协助诊断中枢神经系统神经精神狼疮。虽然病程中脑脊液各项指标可或多或少呈现异常，但白细胞计数和蛋白定量升高不明显，葡萄糖和氯化物水平尚在正常值范围。提示中枢神经系统神经精神狼疮患者脑脊液改变不明显，有助于与颅内感染的鉴别诊断。糖皮质激素和免疫抑制剂对治疗中枢神经系统神经精神狼疮有显著疗效。

关键词: 狼疮血管炎, 中枢神经系统, 血清学, 脑脊髓液, 磁共振成像, 脑电描记术

Abstract: Background Neuropsychiatric systemic lupus erythematosus (NP-SLE) presents with a wide variety of clinical manifestations, which is often difficult to diagnose with a high mortality. This study aims to investigate the clinical features of NP-SLE involving the central nervous system (CNS) and the differential diagnoses between CNS NP-SLE and intracranial infections. Methods The clinical manifestations, serum immunological features, cerebrospinal fluid (CSF) examinations (including intracranial pressure, leukocyte count, protein, glucose and chloride), CT and (or) MRI and electroencephalogram (EEG) data of 23 NP-SLE patients with CNS involved were retrospectively reviewed. Results Nine patients presented with diffuse manifestations, while 14 patients presented with focal manifestations. Serum analysis showed the positive rates of immunoglobulins anti-nuclear antibody (ANA), anti-double stranded DNA antibody (dsDNA), anti-Sm, anti-ribosmal P protein, anti-SSA and anti-SSB antibodies were 21/22, 7/22, 1/14, 2/14, 9/14 and 3/14 respectively. Patients with decreased serum C3 accounted for 14/20 while patients with decreased serum C4 accounted for 5/20. Besides, patients with increased CSF leukocyte count and microalbumin took up 5/12 and 7/12, while patients with decreased glucose and chloride levels took up 5/12 and 6/12. All 23 patients presented abnormal CT and (or) MRI and 6 patients presented abnormal EEG. Conclusion Serum immunological levels, CT and (or) MRI and EEG examinations contributed to the diagnosis of NP-SLE involving CNS. Although CSF analyses were slightly abnormal, the increase of leukocyte count and average microalbumin was not obvious, and the mean values of glucose and chloride were in the normal range, suggesting that the CSF examinations were helpful for the differential diagnoses from intracranial infections. Glucocorticoids and immunosuppressive drugs were remarkably effective for CNS NP-SLE patients.

Key words: Lupus vasculitis, central nervous system , Serology, Cerebrospinal fluid, Magnetic resonance imaging, Electroencephalography