摘要
目的分析探讨结缔组织病(CTD)合并长节段横贯性脊髓炎(LETM)患者的临床特征与预后。方法选2006年1月至2016年12月北京协和医院神经内科和风湿免疫科住院的40例合并LETM的CTD患者,根据是否出现视神经脊髓炎谱系疾病(NMOSD),将患者分为CTD-LETM-NMOSD者和CTD-LETM-非NMOSD者,回顾性分析患者一般资料、临床特征、自身抗体、影像学表现、治疗、预后和转归,采用Kaplan-Meier曲线分析疾病复发率。结果40例患者中,系统性红斑狼疮24例,原发性干燥综合征16例。CTD-LETM-NMOSD者28例(70.0%),CTD-LETM-非NMOSD者12例(30.0%)。CTD-LETM-NMOSD者抗SSA抗体阳性率(75.0%比5/12,P<0.05)、抗水通道蛋白4(AQP4)抗体(AQP4-IgG)阳性率(89.3%比0,P<0.05)显著高于CTD-LETM-非NMOSD者。两者间CTD类型、CTD病程、脊髓炎首发比例、抗Sm抗体、抗U1核糖核蛋白抗体、抗双链DNA抗体、抗SSB抗体、抗核糖体核蛋白P抗体、抗磷脂抗体、脑脊液特异性寡克隆区带及影像学特点均差异无统计学意义。Kaplan-Meier曲线显示,CTD-LETM-NMOSD者疾病复发率高于CTD-LETM-非NMOSD者[75.0%(21/28)比3/12,P<0.01]。结论CTD合并LETM可分为两个亚型,即NMOSD和非NMOSD,前者抗SSA抗体、抗AQP4抗体阳性率及复发率均高于后者,提示其发病机制可能存在差异,前者可能是CTD与NMOSD共病,而后者可能是CTD继发神经系统损害。
Objective Longitudinally extensive transverse myelitis(LETM)could be seen in patients with connective tissue disease(CTD),especially systemic lupus erythematosus(SLE)or primary Sjögren′s syndrome(pSS).Some patients are combined with neuromyelitis optica spectrum disorders(NMOSD)(termed CTD-LETM-NMOSD)while others without(termed CTD-LETM-non-NMOSD).The aim of this study is to compare the clinical characteristics of CTD-LETM-NMOSD patients to CTD-LETM-non-NMOSD patients.Methods We retrospectively collected data from 40 CTD patients with LETM who were admitted to the Department of Neurology or Rheumatology at Peking Union Medical College Hospital from Jan,2006 to Dec,2016.They were divided into CTD-LETM-NMOSD and CTD-LETM-non-NMOSD two groups.Demographic characteristics,clinical and laboratory features were obtained from the database.Relapse rates and clinical outcome were analyzed by Kaplan-Meier method.Results Among 40 patients with CTD,28(70.0%)were NMOSD while 12(30.0%)were not.The positivity rates of anti-SSA,antibodies to aquaporin-4(anti-AQP4)were significantly higher in patients with NMOSD than those in patients with non-NMOSD(P<0.05).Age,gender,clinical features,disease duration,anti-double-stranded DNA antibody,anti-ribosomal P antibody,antiphospholipid antibodies,expanded disability status scale(EDSS)scores,and magnetic resonance imaging(MRI)features were all comparable between two groups.CTD-NMOSD patients had significantly higher disease relapse rate(75.0%vs.3/12,P<0.01).Conclusion Anti-SSA and anti-AQP4 positivity is associated with NMOSD and higher relapse rates,which suggests that NMOSD in CTD-LETM patients may represent distinct characteristics and pathogenesis from patients with CTD-LETM-non NMOSD.
作者
张遥
赵久良
尹翮翔
徐雁
曾小峰
崔丽英
Zhang Yao;Zhao Jiuliang;Yin Hexiang;Xu Yan;Zeng Xiaofeng;Cui Liying(Center of Multiple Sclerosis,Department of Neurology,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100730,China;Department of Rheumatology and Clinical Immunology,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,National Clinical Research Center for Dermatologic and Immunologic Diseases,Beijing 100730,China)
出处
《中华内科杂志》
CAS
CSCD
北大核心
2021年第5期453-458,共6页
Chinese Journal of Internal Medicine
基金
中国医学科学院罕见病研究中心(2016ZX310174-4)
共享阳光-重大疾病临床科研合作项目(FW-HXKT2020022400217)。
