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自身免疫抗体阳性的视神经脊髓炎谱系疾病(NMOSD)患者的临床特征及预后

Clinical features and prognosis of NMOSD patients with positive autoimmune antibodies.

作者信息

Liu Yutao, Liu Yang, Yuan Han, Wang Limei

机构信息

Department of Neurology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou University, Zhengzhou, China.

出版信息

Front Neurol. 2025 Aug 26;16:1634127. doi: 10.3389/fneur.2025.1634127. eCollection 2025.

Abstract

BACKGROUND

This study aimed to compare clinical outcomes in NMOSD patients with non-AQP4-IgG autoantibodies, specifically anti-connective tissue disease antibodies (anti-CTD Abs) and antithyroid antibodies (ATAbs), to evaluate their impact on disease severity and prognosis.

METHODS

A retrospective analysis was conducted using data from NMOSD inpatients with follow-up periods ≥180 days, stratified by antibody status: anti-CTD Abs (+)/(-), ATAbs (+)/(-), or double positivity (+)/(-). The primary outcomes included relapse rates, Expanded Disability Status Scale (EDSS) scores, and survival outcomes.

RESULTS

(1) Anti-CTD Abs (+): higher proportion of female patients, increased relapse frequency; decreased red blood cell (RBC) count and aspartate aminotransferase (AST) levels. (2) ATAbs (+): greater incidence of acute brainstem syndrome (ABS); reduced peripheral leukocyte, neutrophil, and lymphocyte counts; elevated serum urea levels. (3) Double (+): marked female predominance, higher incidence of ABS, decreased RBC count, hemoglobin (Hb) levels, and cerebrospinal fluid (CSF) chloride concentration; elevated serum urea. (4) AQP4-IgG association: AQP4-IgG-positive patients were more frequently female, with higher prevalence of anti-CTD Abs positivity but lower prevalence of ATAbs positivity. (5) Prognostic analysis: both double-positive and single-antibody-positive groups showed higher disability (EDSS ≥4.0/≥6.0) compared with antibody-negative patients, although no significant differences were observed between the two single-antibody subgroups. (6) Multivariate analysis identified combined antibody positivity (OR = 16.292), baseline EDSS score (OR = 3.179), and age at onset (OR = 1.052) as independent predictors of poor clinical outcomes.

CONCLUSION

Routine screening for anti-CTD Abs and ATAbs in NMOSD patients may aid in assessing disease severity and prognosis. Patients with double positivity represent a high-risk subgroup requiring aggressive therapeutic strategies to prevent severe disability.

摘要

背景

本研究旨在比较非水通道蛋白4免疫球蛋白(AQP4-IgG)自身抗体,特别是抗结缔组织病抗体(抗CTD抗体)和抗甲状腺抗体(ATAbs)的视神经脊髓炎谱系障碍(NMOSD)患者的临床结局,以评估它们对疾病严重程度和预后的影响。

方法

使用随访期≥180天的NMOSD住院患者数据进行回顾性分析,按抗体状态分层:抗CTD抗体(+)/(-)、ATAbs(+)/(-)或双阳性(+)/(-)。主要结局包括复发率、扩展残疾状态量表(EDSS)评分和生存结局。

结果

(1)抗CTD抗体(+):女性患者比例更高,复发频率增加;红细胞(RBC)计数和天冬氨酸转氨酶(AST)水平降低。(2)ATAbs(+):急性脑干综合征(ABS)发生率更高;外周白细胞、中性粒细胞和淋巴细胞计数减少;血清尿素水平升高。(3)双阳性(+):女性明显占优势,ABS发生率更高,RBC计数、血红蛋白(Hb)水平和脑脊液(CSF)氯化物浓度降低;血清尿素升高。(4)AQP4-IgG相关性:AQP4-IgG阳性患者女性更常见,抗CTD抗体阳性患病率更高,但ATAbs阳性患病率更低。(5)预后分析:与抗体阴性患者相比,双阳性和单抗体阳性组均显示更高的残疾率(EDSS≥4.0/≥6.0),尽管两个单抗体亚组之间未观察到显著差异。(6)多变量分析确定联合抗体阳性(OR = 16.292)、基线EDSS评分(OR = 3.179)和发病年龄(OR = 1.052)是临床结局不良的独立预测因素。

结论

对NMOSD患者进行抗CTD抗体和ATAbs的常规筛查可能有助于评估疾病严重程度和预后。双阳性患者代表一个高危亚组,需要积极的治疗策略来预防严重残疾。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/82bd/12417128/a98424cd9346/fneur-16-1634127-g001.jpg

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