伴系统性自身抗体阳性的视神经脊髓炎谱系疾病临床特点分析  

Analysis of the clinical characteristics of neuromyelitis optica spectrum disorders with systemic autoantibodies

作　　者：张锐[1] 王莉[1] 陶永丽[1] 蒋晨阳[1] 刘凯[1] 宋波[1] 许予明[1] ZHANG Rui;WANG Li;TAO Yongli;JIANG Chenyang;LIU Kai;SONG Bo;XU Yuming(The First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院,河南郑州450052

出　　处：《中国实用神经疾病杂志》2024年第8期981-985,共5页Chinese Journal of Practical Nervous Diseases

基　　金：国家卫生健康委重点实验室项目(编号:2020-PT310-010);河南省科技攻关项目(编号:242102310191)。

摘　　要：目的对比分析伴或不伴系统性自身抗体阳性的视神经脊髓炎谱系疾病(NMOSD)患者的临床特点。方法回顾性收集2019-06—2021-06于郑州大学第一附属医院住院诊治的NMOSD患者资料,按是否伴其他非特异性自身抗体分为阳性组和阴性组,比较2组患者的临床特征、实验室检查结果、影像学表现等。结果共收集到265例患者的临床资料,男女比例1∶4.5,阳性组患者占61.89%(164/265)。与阴性组患者相比,阳性组女性占比更高(90.24%比68.32%,P<0.01),AQP4阳性率更高(84.15%比66.34%,P<0.01),阳性组与阴性组随访扩展残疾状态量表(EDSS)评分分别为4.0(0,6)、2.0(0,3)(P<0.01),阳性组与阴性组脊髓受累阶段数分别为7.5(5,11)、6(4,8.3)(P=0.027),阳性组脑脊液24 h鞘内合成率7.63(3.10,16.17),阴性组为3.45(0.61,11.02)(P<0.01)。结论伴系统性自身抗体阳性的NMOSD患者脊髓受累节段更长,脑脊液炎症反应更明显,随访EDSS评分更高,非特异性自身抗体的存在可能预示不良预后。Objective To compare and analyze the differences in clinical characteristics between NMOSD patients with and without systemic autoantibodies.Methods The clinical features,laboratory and imaging findings of NMOSD patients seen at the First Affiliated Hospital of Zhengzhou University from June 2019 to June 2021 were recorded.All the patients were divided into two groups according to the status of systemic autoantibody and the corresponding data were analyzed.Results The male to female ratio was 1∶4.5 of the 265 patients collected,and up to 61.89%(164/265)were positive for non-organ-specific autoantibodies.Compared with patients in the negative group,patients in the positive group demonstrated higher percentage of females(90.24%vs 68.32%,P<0.01),higher rate of AQP4 positivity(90.24%vs 68.32%,P<0.01).The follow-up extended disability status scale(EDSS)score was 4.0(0,6)in the positive group and 2.0(0,3)in the negative group(P<0.01),the number of segments of spinal cord involvement was 7.5(5,11)in the positive group and 6(4,8.3)in the negative group(P=0.027),and the 24-hour intrathecal synthesis rate of CSF was 7.63(3.10,16.17)in the positive group and 3.45(0.61,11.02)in the negative group(P<0.01).Conclusion NMOSD patients with systemic autoantibodies have longer segments of spinal cord involvement,more severe and disturbed immune status,and higher follow-up EDSS score.The presence of nonspecific autoantibodies in NMOSD patients may portend a poor prognosis.

关 键 词：视神经脊髓炎谱系疾病 自身抗体 临床特点 影像学表现 炎症反应 预后 

分 类 号：R744.5[医药卫生—神经病学与精神病学]