Racial differences in neuromyelitis optica spectrum disorder

Racial differences in neuromyelitis optica spectrum disorder
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DOI:
10.1212/wnl.0000000000006574
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发表时间:
2018-11-27
期刊:
影响因子:
9.9
通讯作者:
Kim, Ho Jin
Kim, Ho Jin
中科院分区:
医学1区
文献类型:
--
作者:
Kim, Su-Hyun;Mealy, Maureen A.;Kim, Ho Jin

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目的探讨视神经脊髓炎谱系障碍的种族差异。方法对来自丹麦、德国、韩国、英国、美国和泰国6个中心的603例抗水通道蛋白-4抗体阳性患者进行回顾性分析。亚裔和非裔美国人/非裔欧洲人患者的发病年龄比高加索人患者更早(平均分别为36岁、33岁和44岁;p<0.001)。在病程中,高加索患者(23%)脑/脑干受累的发生率低于亚洲患者(42%)和非裔美国人/欧洲人患者(38%)(p<0.001)。发病时严重发作(视力最低点为6.0时),非裔美国人/非欧洲人(58%)比亚洲人(46%)和高加索人(38%)患者更常见(p=0.005)。在多变量分析中,发病年龄较大、免疫抑制治疗前后发作次数较多,而种族不是严重运动残疾的独立预测因素。结论对一项大型国际队列研究的回顾显示,种族影响临床表型、发病年龄和发作严重程度,但总体预后最依赖于早期有效的免疫抑制治疗。
ObjectiveWe aimed to evaluate racial differences in the clinical features of neuromyelitis optica spectrum disorder.MethodsThis retrospective review included 603 patients (304 Asian, 207 Caucasian, and 92 Afro-American/Afro-European), who were seropositive for anti-aquaporin-4 antibody, from 6 centers in Denmark, Germany, South Korea, United Kingdom, United States, and Thailand.ResultsMedian disease duration at last follow-up was 8 years (range 0.3-38.4 years). Asian and Afro-American/Afro-European patients had a younger onset age than Caucasian patients (mean 36, 33, and 44 years, respectively; p < 0.001). During the disease course, Caucasian patients (23%) had a lower incidence of brain/brainstem involvement than Asian (42%) and Afro-American/Afro-European patients (38%) (p < 0.001). Severe attacks (visual acuity = 6.0 at nadir) at onset occurred more frequently in Afro-American/Afro-European (58%) than in Asian (46%) and Caucasian (38%) patients (p = 0.005). In the multivariable analysis, older age at onset, higher number of attacks before and after immunosuppressive treatment, but not race, were independent predictors of severe motor disabilities at last follow-up.ConclusionA review of a large international cohort revealed that race affected the clinical phenotype, age at onset, and severity of attacks, but the overall outcome was most dependent on early and effective immunosuppressive treatment.