Clinical and immunological differences between primary and autoimmune-associated neuromyelitis optica spectrum disorders: a retrospective study
Introduction 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. Addition...
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BMJ Publishing Group
2025-04-01
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| Series: | Lupus Science and Medicine |
| Online Access: | https://lupus.bmj.com/content/12/1/e001491.full |
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| author | Hsien-Tzung Liao Chang-Youh Tsai Wei-Sheng Chen Ying-Ying Yang Hung-Cheng Tsai Yi-Syuan Sun Wan-Hao Tsai De-Feng Huang |
| author_facet | Hsien-Tzung Liao Chang-Youh Tsai Wei-Sheng Chen Ying-Ying Yang Hung-Cheng Tsai Yi-Syuan Sun Wan-Hao Tsai De-Feng Huang |
| author_sort | Hsien-Tzung Liao |
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| description | Introduction 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.Methods 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.Results 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.Discussion 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. |
| format | Article |
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| institution | OA Journals |
| issn | 2053-8790 |
| language | English |
| publishDate | 2025-04-01 |
| publisher | BMJ Publishing Group |
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| series | Lupus Science and Medicine |
| spelling | doaj-art-2f8869d9fd0c4b019aebbaf35e9ededd2025-08-20T02:12:30ZengBMJ Publishing GroupLupus Science and Medicine2053-87902025-04-0112110.1136/lupus-2024-001491Clinical and immunological differences between primary and autoimmune-associated neuromyelitis optica spectrum disorders: a retrospective studyHsien-Tzung Liao0Chang-Youh Tsai1Wei-Sheng Chen2Ying-Ying Yang3Hung-Cheng Tsai4Yi-Syuan Sun5Wan-Hao Tsai6De-Feng Huang7Faculty of Medicine, School of Medicine, National Yang Ming Chiao Tung University Taipei Campus, Taipei, TaiwanFaculty of Medicine, School of Medicine, National Yang Ming Chiao Tung University Taipei Campus, Taipei, TaiwanDivision of Allergy, Immunology and Rheumatology, Taipei Veterans General Hospital, Taipei, TaiwanDivision of Gastroenterology and Hepatology, Taipei Veterans General Hospital, Taipei, TaiwanDivision of Allergy, Immunology and Rheumatology, Taipei Veterans General Hospital, Taipei, TaiwanDivision of Allergy, Immunology and Rheumatology, Taipei Veterans General Hospital, Taipei, TaiwanDivision of Immunology and Rheumatology, Fu Jen Catholic University Hospital, New Taipei, TaiwanFaculty of Medicine, School of Medicine, National Yang Ming Chiao Tung University Taipei Campus, Taipei, TaiwanIntroduction 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.Methods 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.Results 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.Discussion 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.https://lupus.bmj.com/content/12/1/e001491.full |
| spellingShingle | Hsien-Tzung Liao Chang-Youh Tsai Wei-Sheng Chen Ying-Ying Yang Hung-Cheng Tsai Yi-Syuan Sun Wan-Hao Tsai De-Feng Huang Clinical and immunological differences between primary and autoimmune-associated neuromyelitis optica spectrum disorders: a retrospective study Lupus Science and Medicine |
| title | Clinical and immunological differences between primary and autoimmune-associated neuromyelitis optica spectrum disorders: a retrospective study |
| title_full | Clinical and immunological differences between primary and autoimmune-associated neuromyelitis optica spectrum disorders: a retrospective study |
| title_fullStr | Clinical and immunological differences between primary and autoimmune-associated neuromyelitis optica spectrum disorders: a retrospective study |
| title_full_unstemmed | Clinical and immunological differences between primary and autoimmune-associated neuromyelitis optica spectrum disorders: a retrospective study |
| title_short | Clinical and immunological differences between primary and autoimmune-associated neuromyelitis optica spectrum disorders: a retrospective study |
| title_sort | clinical and immunological differences between primary and autoimmune associated neuromyelitis optica spectrum disorders a retrospective study |
| url | https://lupus.bmj.com/content/12/1/e001491.full |
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