School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.
School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran; Student Research Committee, Isfahan University of Medical Sciences, Isfahan, Iran.
Mult Scler Relat Disord. 2024 Oct;90:105843. doi: 10.1016/j.msard.2024.105843. Epub 2024 Aug 23.
Neuromyelitis Optica Spectrum Disorders (NMOSD) is a neuroinflammatory condition characterized by optic neuritis and transverse myelitis. While the current approach to NMOSD focuses on relapse-associated worsening (RAW), recent evidence indicates Relapse-Independent Disease Activity (RIDA) in patients.
Databases including Embase, PubMed, Scopus, and Web of Sciences were systematically searched up to December 2023. No restrictions were applied. Inclusion criteria focused on studies reporting evidence of RIDA in NMOSD patients. Data extraction involved details such as study title, author, participant characteristics, treatment, evaluation methods, positive findings according to RIDA, and prevalence of findings in NMOSD patients. This study is conducted following the PRISMA guidelines with a registered protocol on PROSPERO (ID = CRD42023492352).
Of 802 studies, 38 were included in the systematic review, covering 1881 NMOSD patients. AQP4-IGg status was positive in 90.6 % of the patients. Ocular findings indicative of RIDA were reported in 23 studies, including thinning of GCIPL, RNFL, GCC, and GCL layers, foveal and macular shape and volume abnormalities, vessel loss, and visual evoked potentials (VEPs) abnormalities. MRI findings supporting the RIDA were reported in 13 studies, including new lesion incidence and brain and spinal cord atrophy. Serum and CSF RIDA-supporting findings were reported in five studies, including elevation in sGFAP and sNFL. Biopsies and autopsies suggested inflammatory processes in relapse-free patients in 2 studies. The predominant manifestation of RIDA in NMOSD was identified in the visual system, suggesting the impaired retinal glial cells like Müller cells during the relapse-free period in NMOSD.
Our systematic review provides valuable insights into RIDA in NMOSD. Establishing guidelines for the diagnosis and treatment of RIDA is crucial. Further studies are needed to provide robust evidence on RIDA in NMOSD patients.
视神经脊髓炎谱系疾病（NMOSD）是一种以视神经炎和横贯性脊髓炎为特征的神经炎症性疾病。虽然目前 NMOSD 的治疗重点是与复发相关的恶化（RAW），但最近的证据表明 NMOSD 患者存在与复发无关的疾病活动（RIDA）。
系统检索了 Embase、PubMed、Scopus 和 Web of Sciences 等数据库，检索时间截至 2023 年 12 月。未对数据库进行任何限制。纳入标准主要包括报告 NMOSD 患者存在 RIDA 证据的研究。数据提取内容包括研究标题、作者、参与者特征、治疗方法、评估方法、根据 RIDA 得出的阳性发现以及 NMOSD 患者的阳性发现发生率。本研究遵循 PRISMA 指南进行，在 PROSPERO （ID = CRD42023492352）上注册了方案。
在 802 项研究中，有 38 项研究被纳入系统评价，共纳入 1881 例 NMOSD 患者。患者中 AQP4-IGg 阳性率为 90.6%。23 项研究报告了提示 RIDA 的眼部发现，包括 GCIPL、RNFL、GCC 和 GCL 层变薄、黄斑和黄斑形状和体积异常、血管丢失和视觉诱发电位（VEP）异常。13 项研究报告了支持 RIDA 的 MRI 发现，包括新发病灶发生率和脑和脊髓萎缩。5 项研究报告了支持 RIDA 的血清和 CSF 发现，包括 sGFAP 和 sNFL 升高。2 项研究通过活检和尸检表明，在无复发期间，缓解期患者存在炎症过程。NMOSD 中 RIDA 的主要表现是在视觉系统中，这表明在 NMOSD 无复发期间，视网膜神经胶质细胞（如 Müller 细胞）受损。
本系统评价为 NMOSD 中的 RIDA 提供了有价值的见解。制定 RIDA 的诊断和治疗指南至关重要。需要进一步的研究为 NMOSD 患者的 RIDA 提供更有力的证据。
