Tsai Hung-Cheng, Sun Yi-Syuan, Chen Wei-Sheng, Tsai Wan-Hao, Huang De-Feng, Yang Ying-Ying, Liao Hsien-Tzung, Tsai Chang-Youh
Division of Allergy, Immunology and Rheumatology, Taipei Veterans General Hospital, Taipei, Taiwan.
Faculty of Medicine, School of Medicine, National Yang Ming Chiao Tung University Taipei Campus, Taipei, Taiwan.
Lupus Sci Med. 2025 Apr 14;12(1):e001491. doi: 10.1136/lupus-2024-001491.
Neuromyelitis optica spectrum disorder (NMOSD) is a rare immune-mediated disease affecting the spinal cord and optic nerves. While NMOSD has been widely studied, limited data exist on the subset associated with autoimmune diseases (AD-NMOSD), particularly in Taiwanese patients. Additionally, relapse and prognostic factors in AD-NMOSD remain unclear.
We retrospectively analysed 71 NMOSD cases diagnosed between 2008 and 2023 at Taipei Veterans General Hospital. Clinical features, laboratory findings, autoimmune comorbidities, imaging and treatments were examined. Patients were stratified by relapse status and the presence of severe sequelae.
Among 71 NMOSD cases, 26 (37%) patients had AD-NMOSD. While no significant differences were observed in the number or severity of relapses and sequelae between AD-NMOSD and primary (p)-NMOSD, patients with AD-NMOSD exhibited lower white blood cell counts, haemoglobin, platelet counts, immunoglobulin G and C reactive protein levels. Specific risk factors for relapse in AD-NMOSD included onset age under 50 years, concurrent SLE and a longer duration of SLE before NMOSD presentation. In both AD-NMOSD and p-NMOSD, more relapses were associated with severe neurological sequelae. Although relapse-free survival did not differ significantly between the two groups, patients with AD-NMOSD tended to have a longer period without severe sequelae.
Taiwanese patients with AD-NMOSD show distinct laboratory characteristics compared with those without autoimmune diseases. Younger age and longer disease duration are key risk factors for relapses, which are linked to more severe neurological sequelae. Despite various treatments, no significant differences were found in relapse rates or sequelae severity, highlighting the need for personalised management strategies.
视神经脊髓炎谱系障碍（NMOSD）是一种罕见的免疫介导性疾病，会影响脊髓和视神经。虽然NMOSD已得到广泛研究，但关于与自身免疫性疾病相关的亚组（AD-NMOSD）的数据有限，尤其是在台湾患者中。此外，AD-NMOSD的复发和预后因素仍不清楚。
我们回顾性分析了2008年至2023年在台北荣民总医院诊断的71例NMOSD病例。检查了临床特征、实验室检查结果、自身免疫性合并症、影像学和治疗情况。根据复发状态和严重后遗症的存在对患者进行分层。
在71例NMOSD病例中，26例（37%）患者患有AD-NMOSD。虽然AD-NMOSD与原发性（p）-NMOSD在复发次数和严重程度以及后遗症方面未观察到显著差异，但AD-NMOSD患者的白细胞计数、血红蛋白、血小板计数、免疫球蛋白G和C反应蛋白水平较低。AD-NMOSD复发的特定危险因素包括发病年龄在50岁以下、并发系统性红斑狼疮（SLE）以及在NMOSD出现前SLE病程较长。在AD-NMOSD和p-NMOSD中，更多的复发与严重的神经后遗症相关。虽然两组之间无复发生存期无显著差异，但AD-NMOSD患者无严重后遗症的时间往往更长。
与无自身免疫性疾病的台湾患者相比，患有AD-NMOSD的患者表现出不同的实验室特征。年龄较小和病程较长是复发的关键危险因素，且与更严重的神经后遗症有关。尽管进行了各种治疗，但在复发率或后遗症严重程度方面未发现显著差异，这突出了个性化管理策略的必要性。
