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预测视神经脊髓炎谱系障碍(NMOSD)发病五年后状况的社会人口统计学和疾病相关指标

Sociodemographic and Illness-Related Indicators to Predict the Status of Neuromyelitis Optica Spectrum Disorder (NMOSD) Five Years after Disease Onset.

作者信息

Sadeghi-Bahmani Dena, Barzegar Mahdi, Mirmosayyeb Omid, Vaheb Saeed, Nehzat Nasim, Shaygannejad Vahid, Brand Serge

机构信息

Department of Psychology, University of Stanford, Stanford, CA 94305, USA.

Psychiatric Clinics, Center for Affective, Stress and Sleep Disorders, University of Basel, 4002 Basel, Switzerland.

出版信息

J Clin Med. 2022 Jan 29;11(3):734. doi: 10.3390/jcm11030734.

Abstract

BACKGROUND

Neuromyelitis Optica Spectrum Disorder (NMOSD) is an autoimmune demyelinating disease of the central nervous system. Currently, no factors have been identified to predict the long-term course of NMOSD. To counter this, we analyzed data of 58 individuals with NMOSD at disease onset and about five years later.

METHODS

Medical records of 58 individuals with NMOSD (mean age: 31.13 years at disease onset; 86.2% female) were retrospectively analyzed. At baseline, a thorough medical and disease-related examination was performed; the same examination was repeated about five years later at follow-up, including treatment-related information. Mean outcome measure was the difference in EDSS (Expanded Disease Severity Scale) scores between baseline and follow-up.

RESULTS

Mean disease duration was 4.67 years. Based on the differences of the EDSS scores between baseline and follow-up, participants were categorized as improving ( = 39; 67.2%), unchanged ( = 13; 22.4%) and deteriorating ( = 6; 10.3%). Deteriorating was related to a higher progression index, and a higher number of attacks, while the annualized relapse rate reflecting the number of attacks per time lapse did not differ between the three groups. Improving was related to a higher intake of rituximab, and to a higher rate of seropositive cases. Unchanged was related to a lower rate of seropositive cases. Factors such as age, gender, somatic and psychiatric comorbidities, symptoms at disease onset, relapse rates, number and location of cervical plaques, or brain plaques and thoracolumbar plaques at baseline did not differ between those improving, deteriorating or remaining unchanged.

CONCLUSIONS

Among a smaller sample of individuals with NMOSD followed-up about five years later, individuals deteriorating over time reported a higher progression index, while the annualized relapse rate was unrelated to the progress of disease. Overall, it appears that the course of NMOSD over a time lapse of about five years after disease onset is highly individualized. Accordingly, treatment regimen demands a highly individually tailored approach.

摘要

背景

视神经脊髓炎谱系障碍(NMOSD)是一种中枢神经系统的自身免疫性脱髓鞘疾病。目前,尚未发现可预测NMOSD长期病程的因素。为解决这一问题,我们分析了58例NMOSD患者疾病初发时及大约五年后的相关数据。

方法

对58例NMOSD患者(疾病初发时平均年龄:31.13岁;86.2%为女性)的病历进行回顾性分析。在基线时,进行了全面的医学及疾病相关检查;大约五年后的随访时重复相同检查,包括与治疗相关的信息。平均结局指标为基线与随访时扩展残疾状态量表(EDSS)评分的差异。

结果

平均病程为4.67年。根据基线与随访时EDSS评分的差异,参与者被分为改善组(n = 39;67.2%)、不变组(n = 13;22.4%)和恶化组(n = 6;10.3%)。恶化与更高的进展指数及更多的发作次数相关,而反映每次时间间隔发作次数的年化复发率在三组之间无差异。改善与更高的利妥昔单抗摄入量及更高的血清学阳性率相关。不变与更低的血清学阳性率相关。改善、恶化或不变的患者在年龄、性别、躯体和精神共病、疾病初发时的症状、复发率、颈部斑块的数量和位置、或基线时的脑斑块及胸腰椎斑块等因素方面无差异。

结论

在约五年后随访的一小部分NMOSD患者中,随时间恶化的患者报告有更高的进展指数,而年化复发率与疾病进展无关。总体而言,疾病初发后约五年内NMOSD的病程似乎高度个体化。因此,治疗方案需要高度个体化定制。

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