视神经脊髓炎谱系疾病全身表现分析及其机制探讨

Systemic manifestations and pathogenesis exploration of neuromyelitis optica spectrum disorders

目的研究视神经脊髓炎谱系疾病合并中枢神经系统以外受累脏器或组织的免疫学改变。方法回顾分析56例视神经脊髓炎谱系疾病患者(视神经脊髓炎33例、非视神经脊髓炎23例)临床资料,逐一记录是否合并其他自身免疫性疾病,并行血清免疫学检测。结果视神经脊髓炎患者伴桥本甲状腺炎2例,以及系统性红斑狼疮和干燥综合征、哮喘、甲状腺功能亢进、风湿性关节炎和虹膜睫状体炎各1例。血清免疫学指标异常检出率较高者分别为甲状腺功能异常(10/17例)、抗核抗体阳性(14/28例)和补体C3阳性(8/19例),2例于分娩后发病。结论视神经脊髓炎谱系疾病患者可合并多种自身免疫性疾病,同时存在多种自身抗体,可能与视神经脊髓炎谱系疾病的发病有关。此外,妊娠期或分娩可能加重患者病情。

Objective To investigate the immunological abnormalities beyond central nervous system （CNS） associated with neuromyelitis optica spectrum disorders （NMOSDs）. Methods Clinical data of 56 patients with NMOSDs from January 2010 to December 2013 enrolled in Department of Neurology at Peking University People＇s Hospital were analyzed retrospectively. All patients were divided into 2 groups： neuromyelitis optica （N = 33） and non-neuromyelitis optiea （N = 23）. Each patient underwent detailed physical examination including internal medicine and nervous system. Records of complicated autoimmune diseases as well as scoring of Expanded Disability Status Scale （EDSS） were used to evaluate disease severity. Part of patients received detection of multiple immunological indicators. Results In all patients with NMOSDs, there were 3 cases with Hashimoto thyroiditis, one case with systemic lupus erythematosus （SLE） and Sjogren＇s syndrome （SS）, asthma, hyperthyroidism, rheumatoid arthritis and iridocyclitis, respectively. In patients whose immunological indices were available, the first three abnormal immunological changes were abnormal thyroid function （10/17）, positive anti-nuclear antibody （14/28） and positive complement 3 （8/19）. In addition, NMOSDs got worsen in 2 cases after delivery. Conclusions NMOSDs coexist with many kinds of autoimmune diseases and multiple autoantibodies. The above- mentioned autoimmunity may be related to the pathogenesis of NMOSDs. Besides, pregnancy or delivery may aggravate the disease severity of NMOSDs.