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自身免疫性合并症对成人水通道蛋白4视神经脊髓炎谱系障碍(AQP4-NMOSD)和髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)发病、发作及恢复的影响

The impact of autoimmune comorbidities on the onset attack recovery in adults with AQP4-NMOSD and MOGAD.

作者信息

Samadzadeh Sara, Chan Fiona, Francis Anna, Sani Layana, Paul Friedemann, Asgari Nasrin, Leite M Isabel, Geraldes Ruth, Palace Jacqueline

机构信息

Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK.

Institute of Regional Health Research and, Institute of Molecular Medicine, University of Southern Denmark, Odense, Denmark.

出版信息

J Neurol. 2025 Jun 10;272(7):453. doi: 10.1007/s00415-025-13180-3.

DOI:10.1007/s00415-025-13180-3
PMID:40495001
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12152089/
Abstract

BACKGROUND

Aquaporin-4 neuromyelitis optica spectrum disorder (AQP4-NMOSD) often coexists with other autoimmune diseases (AIDs), whereas such comorbidities are less common in myelin oligodendrocyte glycoprotein antibody disease (MOGAD). This study investigates the impact of additional AIDs on early relapse recovery and disability in patients with AQP4-NMOSD and MOGAD.

METHODS

This retrospective study included patients aged > 16 years with AQP4-NMOSD (n = 175) or MOGAD (n = 221), who were followed at a nationally commissioned Oxford service and categorized based on the presence of at least one AID. Outcomes included recovery from the onset attack, visual recovery after the first optic neuritis (ON) attack (≥ 6 months post attack), time to first relapse and time to death. Incomplete visual recovery was defined as visual acuity worse than LogMAR 0.1. Optical coherence tomography (OCT) assessed retinal nerve fiber layer thickness and ganglion cell-inner plexiform layer volume in a subset.

RESULTS

In the AQP4-NMOSD cohort, 28% (n = 49) had at least one AID, compared to 11.3% (n = 25) in the MOGAD cohort (p < 0.001), with thyroid disease constituting the majority of these cases in both groups. In MOGAD, the median age of first attack was significantly higher in the AID group (46 years; IQR: 35-56) than in the non-AID group (35 years; IQR: 28-47) (p = 0.004), a difference that was not observed in the AQP4-NMOSD cohort. In both the AQP4-NMOSD (n = 175) and the MOGAD (n = 221) cohorts, age was a significant predictor of outcome in univariate analyses (AQP4-NMOSD: OR = 0.96 per year, 95% CI: 0.94-0.98, p < 0.001; MOGAD: OR = 0.97 per year, 95% CI: 0.94-0.99, p = 0.008). No significant differences were observed in clinical or visual recovery rates between AID and non-AID patients in either cohort. There were no statistically significant differences observed between AID and non-AID cohorts for clinical or visual recovery outcomes. Similarly, AID status did not influence time to relapse (AQP4-NMOSD: HR = 1.0, 95% CI: 0.63-1.58, p = 0.99; MOGAD: HR = 0.78, 95% CI: 0.40-1.52, p = 0.47) or time to death (AQP4-NMOSD: HR = 0.5, 95% CI: 0.18-1.36, p = 0.28). OCT analysis revealed no significant differences in retinal parameters between AID and non-AID groups in both cohorts.

CONCLUSIONS

Additional autoimmune diseases are unlikely to significantly affect clinical or visual outcomes in early attacks in patients with AQP4-NMOSD and MOGAD.

摘要

背景

水通道蛋白4视神经脊髓炎谱系障碍(AQP4-NMOSD)常与其他自身免疫性疾病(AID)共存,而在髓鞘少突胶质细胞糖蛋白抗体病(MOGAD)中这种合并症较少见。本研究调查了合并其他AID对AQP4-NMOSD和MOGAD患者早期复发恢复及残疾情况的影响。

方法

这项回顾性研究纳入了年龄大于16岁的AQP4-NMOSD患者(n = 175)或MOGAD患者(n = 221),这些患者在牛津一家全国性委托服务机构接受随访,并根据是否存在至少一种AID进行分类。观察指标包括首次发作后的恢复情况、首次视神经炎(ON)发作后(发作后≥6个月)的视力恢复情况、首次复发时间和死亡时间。不完全视力恢复定义为视力低于LogMAR 0.1。光学相干断层扫描(OCT)在一个亚组中评估视网膜神经纤维层厚度和神经节细胞-内丛状层体积。

结果

在AQP4-NMOSD队列中,28%(n = 49)的患者至少有一种AID,而在MOGAD队列中这一比例为11.3%(n = 25)(p < 0.001),两组中甲状腺疾病在这些病例中占大多数。在MOGAD中,AID组首次发作的中位年龄(46岁;四分位间距:35 - 56岁)显著高于非AID组(35岁;四分位间距:28 - 47岁)(p = 0.004),在AQP4-NMOSD队列中未观察到这种差异。在AQP4-NMOSD队列(n = 175)和MOGAD队列(n = 221)中,年龄在单因素分析中均是结局的显著预测因素(AQP4-NMOSD:OR = 0.96/年,95%置信区间:0.94 - 0.98,p < 0.001;MOGAD:OR = 0.97/年,95%置信区间:0.94 - 0.99,p = 0.008)。在两个队列中,AID患者和非AID患者在临床或视力恢复率方面均未观察到显著差异。AID组和非AID组在临床或视力恢复结局方面也未观察到统计学显著差异。同样,AID状态不影响复发时间(AQP4-NMOSD:HR = 1.0,95%置信区间:0.63 - 1.58,p = 0.99;MOGAD:HR = 0.78,95%置信区间:(0.40 - 1.52),p = 0.47)或死亡时间(AQP4-NMOSD:HR = 0.5,95%置信区间:0.18 - 1.36,p = 0.28)。OCT分析显示,两个队列中AID组和非AID组在视网膜参数方面均无显著差异。

结论

合并其他自身免疫性疾病不太可能显著影响AQP4-NMOSD和MOGAD患者早期发作的临床或视力结局。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c486/12152089/a35d0073d9ca/415_2025_13180_Fig4_HTML.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c486/12152089/753d0090f005/415_2025_13180_Fig2_HTML.jpg
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