作者投稿
专家审稿
编辑办公
邮件订阅
RSS
The Clinical Features of Neuropsychiatric Lupus Erythematosus with Normal Cerebrospinal Fluid
-
摘要:
目的 分析脑脊液检测正常狼疮性脑病(neuropsychiatic lupus erythematosus, NPLE)患者的临床特点, 以提高NPLE的诊断。 方法 2013年1月至2016年12月北京协和医院确诊为NPLE的住院患者95例, 收集患者入院第一次留取的脑脊液标本检测结果, 其中脑脊液正常者18例(研究组), 脑脊液异常(压力、细胞数、蛋白任何一项指标异常即为脑脊液异常)者77例(对照组)。对两组性别、年龄、入院症状、起病方式、累及系统、炎症学指标、磁共振成像(magnetic resonance imaging, MRI)结果、治疗情况及住院时间等进行统计学分析, 并进一步对18例脑脊液正常NPLE患者的年龄分布、系统性红斑狼疮(systemic lypus erythematosus, SLE)病程、治疗方案及MRI结果进行描述性分析。 结果 18例脑脊液正常患者中以精神症状起病者10例, 77例脑脊液异常患者中精神症状起病者10例, 两组起病方式差异有统计学意义(55.6%比13.0%, P < 0.05);脑脊液正常患者中NPLE复发患者12例, 脑脊液异常患者中NPLE复发患者20例, 两组比较有统计学意义(66.7%比26.0%, P < 0.05)。18例脑脊液正常NPLE患者中育龄期女性16例(88.9%), 诊断SLE后一年内发病9例(50.0%), 因激素量不足起病17例(94.4%); 9例MRI结果亦正常患者48 h后脑脊液复查均出现异常。 结论 以精神症状起病的SLE患者, 既往有NPLE病史, 入院首次脑脊液即使正常, 也应及时行MRI检查或复查脑脊液, 防止漏诊NPLE。 Abstract:Objective The aim of our study was to analyze clinical features of neuropsychiatric lupus erythematosus(NPLE)with normal cerebrospinal fluid, in order to improve the diagnosis of NPLE. Methods From January 2013 to December 2016, a total of 95 patients diagnosed with NPLE at Peking Union Medical College Hospital were included in our study. Results of the first time exam of cerebrospinal fluid after hospitalization were collected. Cerebrospinal fluid was normal in 18 cases(study group) and abnormal(including any abnormalities in pressure, cell number or protein) in 77 cases(control group). Gender, age, symptoms, mode of onset, involved system, inflammatory markers, magnetic resonance imaging(MRI) findings, dsDNA, treatment, and length of hospitalization were analyzed statistically. Age distribution, systemic lupus erythematosus(SLE) course, treatment, and results of MRI of the 18 patients with NPLE and normal cerebrospinal fluid were further descriptively analyzed. Results Ten of the 18 patients with normal cerebrospinal fluid and 10 of the 77 cases with abnormal cerebrospinal fluid started with mental symptoms. There were significant differences between the two groups(55.6% vs. 13.0%, P < 0.05). Twelve of the study group and 20 of the control group had relapse of NPLE(66.7% vs. 26.0%, P < 0.05). There was no significant difference between the two groups in gender, age, involved system, inflammatory indicators, MRI results, double stranded DNA, treatment, and length of hospitalization(P>0.05). Sixteen of 18 cases of the study group were women at reproductive age(88.9%); 9 occurred in the first year of SLE(50.0%); 17 were initiated from inadequate medication(94.4%); 9 patients with normal MRI findings presented abnormal CSF examination 48 hours later. Conclusion SLE patients with onset of mental symptoms and medical history of NPLE, even with normal cerebrospinal fluid exam, should perform MRI and repeated cerebrospinal fluid exams to avoid the misdiagnosis of NPLE. -
表 1 狼疮性脑病患者研究组和对照组观察指标比较
观察指标 研究组(n=18) 对照组(n=77) P值 性别(男/女, n) 0/18 9/68 0.108 年龄($ \overline x \pm s$, 岁) 36.7±7.6 32.9±8.3 0.135 入院症状(精神症状/神经症状, n) 10/8 10/67 0.002 起病方式(复发/首发, n) 12/6 20/57 0.001 累及系统(累及/未累及, n) 肾脏 8/10 44/33 0.329 血液 12/6 56/21 0.607 心肺 1/17 11/66 0.315 消化 1/17 4/73 0.950 感染 6/12 20/57 0.528 ESR(mm/h) 31.83±22.97 34.28±30.90 0.857 CRP(mg/dl) 13.57±9.03 14.02±10.62 0.123 C3(mg/dl) 0.63±0.37 0.66±0.31 0.579 C4(mg/dl) 0.18±0.23 0.14±0.13 0.913 MRI(阳性/阴性) 9/9 54/23 0.103 双链DNA(阳性/阴性) 4/14 53/24 0.453 治疗方案(激素冲击/冲击+鞘注) 1/17 20/57 0.060 住院天数(d) 31.55±13.89 29.89±12.23 0.808 ESR:红细胞沉降率；CRP:C-反应蛋白；MRI:磁共振成像 
下载: 导出CSV
表 2 18例脑脊液正常NPLE患者临床特点
观察指标(n=18) 例数(%) 年龄分布(岁) 20~30 5(27.8) > 30~40 6(33.3) > 40~50 5(27.8) > 50 2(11.1) SLE病程(年) < 1 9(50.0) 1~5 4(22.2) > 5 5(27.8) 发病时激素治疗情况 减量 12(66.7) 停药 3(16.7) 不规则服药 2(11.1) 正规服药 1(5.6) NPLE：狼疮性脑病
下载: 导出CSV
-
[1] Clark KE, Clark CN, Rahman A. A critical analysis of the tools to evaluate neuropsychiatric lupus[J].Lupus, 2017, 26:504-509. doi: 10.1177/0961203317690242 [2] Zirkzee EJ, Huizinga TW, Bollen EL, et al. Mortality in neuropsychiatric systemic lupus erythematosus(NPSLE)[J]. Lupus, 2014, 23:31-38. doi: 10.1177/0961203313512540 [3] Brey RL, Holliday SL, Saklad AR, et al. Neuropsychiatric syndromes in lupus:prevalence using standardized definitions[J]. Neurology, 2002, 58:1214-1220. doi: 10.1212/WNL.58.8.1214 [4] Bertsias GK, Boumpas DT. Pathogenesis, diagnosis and management of neuropsychiatric SLE manifestations[J]. Nat Rev Rheumatol, 2010, 6:358-367. doi: 10.1038/nrrheum.2010.62 [5] 许怀麟, 陈宜芳, 李希清, 等.系统性红斑狼疮脑病发生的相关因素分析[J].新医学, 2001, 32:144. doi: 10.3969/j.issn.0253-9802.2001.03.012 [6] 陈灏珠, 林果为, 王吉耀.实用内科学[M].北京:人民卫生出版社, 2013:2600-2611. [7] 韩红, 须晋, 李毅, 等.系统性红斑狼疮急症的临床研究[J].临床急症杂志, 2012, 13:176-178. http://www.wanfangdata.com.cn/details/detail.do?_type=perio&id=lcjzzz201203010 [8] The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes[J].Arthritis Rheum, 1999, 42: 599-608. [9] Hanly JG. Diagnosis and management of neuropsychiatric SLE[J].Nat Rev Rheumatol, 2014, 10:338-347. doi: 10.1038/nrrheum.2014.15 [10] Steup-Beekman GM, Zirkzee EJ, Cohen D, et al. Neuropsychiatric manifestations in patients with systemic lupus erythematosus:epidemiology and radiology pointing to an immune-mediated cause[J].Ann Rheum Dis, 2013, 72:S76-S79. doi: 10.1136/annrheumdis-2012-202369 [11] Bhangle SD, Kramer N, Rosenstein ED.Corticosteroid-induced neuropsychiatric disorders:review and contrast with neuropsychiatric lupus[J].Rheumatol Int, 2013, 33:1923. doi: 10.1007/s00296-013-2750-z [12] Sarbu N, Toledano P, Calvo A, et al.Advanced MRI techniques:biomarkers in neuropsychiatric lupus[J].Lupus, 2017, 26:510-516. doi: 10.1177/0961203316674820 [13] 韩红, 于学忠, 李毅等.系统性红斑狼疮急诊就诊的主诉症状[J].协和医学杂志, 2013, 4:279-281. doi: 10.3969/j.issn.1674-9081.2013.03.012 [14] Iizuka N, Okamoto K, Matsushita R, et al. Identification of autoantigens specific for systemic lupus erythematosus with central nervous system involvement[J]. Lupus, 2010, 19:717-726. doi: 10.1177/0961203309357764 [15] 周珣, 李浩.系统性红斑狼疮患者脑脊液的改变规律分析[J].中国实用神经疾病杂志, 2014, 17:79-80. http://www.wanfangdata.com.cn/details/detail.do?_type=perio&id=hnsysjjbzz201422048 -
点击查看大图
表(3)
计量
- 文章访问数: 145
- HTML全文浏览量: 37
- PDF下载量: 4
- 被引次数: 0
