以抽搐或昏迷为临床表现神经精神性狼疮的临床分析

Clinical analysis of neuropsychiatric systemic lupus erythematosus with convulsion or coma as clinical manifestation

目的分析以抽搐或昏迷为主要表现神经精神性狼疮(NPSLE)的临床特点,以利于提高NPSLE患者的诊断。方法收集2013年1月至2016年12月北京协和医院住院确诊为NPSLE的患者92例,其中以抽搐或昏迷为主要表现起病的NPSLE患者27例作为研究组,其余65例为对照组。将两组性别、年龄、是否为首发NPSLE、系统性红斑狼疮(SLE)病史、受累系统(肾、血液、心肺、皮肤黏膜、消化道)、合并感染、脑脊液压力、脑脊液细胞数、脑脊液/血清蛋白比值、脑脊液/血清葡萄糖比值、脑脊液/血清氯比值、红细胞沉降率(ESR)、C-反应蛋白(CRP)、补体C3、补体C4、磁共振成像(MRI)结果、双链DNA抗体、治疗方案、住院时间指标进行比较,发现两者之间的特点。结果研究组脑脊液细胞数明显少于对照组(×106/L:91.84±25.37比279.52±101.12,P<0.01)。研究组皮肤黏膜受累率明显高于对照组〔14.81%(4/27)比1.54%(1/65),P<0.05〕。研究组脑脊液/血清蛋白比值大于对照组(0.12±0.02比0.04±0.01,P<0.05),脑脊液/血清葡萄糖比值明显小于对照组(0.55±0.17比0.70±0.20,P<0.01)。研究组MRI阳性率大于对照组〔81.48%(22/27)比55.38%(36/65),P<0.05〕;其余各指标两组间比较差异均无统计学意义(均P>0.05)。结论脑脊液细胞数少、皮肤黏膜受累、脑脊液/血清蛋白比值增大,而脑脊液/血清葡萄糖比值减少,MRI结果阳性是以抽搐或昏迷为主要临床表现NPSLE患者的临床特点,早期发现并进行干预有利于改善患者预后。

Objective To analyze the clinical features of neuropsychiatric systemic lupus erythematosus (NPSLE) with convulsion or coma as the main manifestation to facilitate the improvement of such patients' diagnosis. Methods Ninety-two patients with NPSLE confirmed in Peking Union Medical Hospital from January 2013 to December 2016 were collected, 27 NPSLE patients with convulsion or coma were in the study group, and the remaining 65 cases were in the control group. The following items in the two groups were compared in order to discover the differences in characteristics between the two groups: including sex, age, the first NPSLE episode or not, history of systemic lupus erythematosus (SLE), kidney, blood, heart, lung, skin mucous membrane, gastrointestinal involvement and co-infection, cerebrospinal fluid (CSF) pressure, cerebrospinal fluid cell count, cerebrospinal fluid/serum protein ratio, cerebrospinal fluid/serum glucose ratio, cerebrospinal fluid/serum chlorine ratio, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), complement 3 (C3), complement 4 (C4), magnetic resonance imaging (MRI) results, double strand deoxyribonucleic acid (ds-DNA) antibody, treatment status and hospitalization days. Results The number of CSF cells in the study group was significantly lower than that in the control group (×106 /L: 91.84±25.37 vs. 279.52±101.12, P < 0.01). The skin mucosa involvement rate in the study group was significantly higher than that in the control group [14.81% (4/27) vs. 1.54% (1/65), P < 0.05]. Cerebrospinal fluid/serum protein ratio was higher in the study group than that in the control group (0.12±0.02 vs. 0.04±0.01, P < 0.05); the cerebrospinal fluid/serum glucose ratio was significantly lower than that in the control group (0.55±0.17 vs. 0.70±0.20, P < 0.01). The positive rate of MRI in the study group was higher than that in the control group [81.48% (22/27) vs. 55.38% (36/65), P < 0.05]; there were no significant differences between the two groups in other indexes (all P > 0.05). Conclusion Few cerebrospinal fluid cells increased involvement of skin mucosa, increased cerebrospinal fluid/serum protein ratio, decreased cerebrospinal fluid glucose/serum glucose ratio and increased MRI positive results were the clinical features of NPSLE patients with convulsion or coma as the clinical manifestation, early detection of this type of patients and early intervention can be beneficial to improve the prognosis.