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血清阳性 NMOSD 的当代管理挑战。

Contemporary management challenges in seropositive NMOSD.

机构信息

Department of Clinical Neurosciences, University of Calgary, Calgary, Canada.

Department of Surgery, University of Calgary, Calgary, Canada.

出版信息

J Neurol. 2022 Oct;269(10):5674-5681. doi: 10.1007/s00415-022-11241-5. Epub 2022 Jul 11.

Abstract

Neuromyelitis optica spectrum disorders (NMOSD) is an inflammatory disorder of the central nervous system that presents unique management challenges. Neurologic disability in NMOSD is directly linked to acute attacks, therefore, relapse prevention is an overarching goal of care. To this end, identifying effective biomarkers that predict relapse onset and severity is of critical importance. As treatment becomes more precision-based and patient-centred, clinicians will need to be familiar with managing circumstances of particular vulnerability for patients with NMOSD, including infection, pregnancy, and the post-partum phase. The discovery of the pathogenic aquaporin-4 Immunoglobulin G (AQP4 IgG) autoantibody almost 20 years ago ultimately distinguished NMOSD as an autoimmune astrocytopathy and helped spearhead recent therapeutic advancements. Targeted therapies, including eculizumab, satralizumab, and inebilizumab, approved for use in aquaporin-4 immunoglobulin G (AQP4 IgG) seropositive patients with NMOSD will likely improve outcomes, but there are formidable costs involved. Importantly, seronegative patients continue to have limited therapeutic options. Moving forward, areas of research exploration should include relapse prevention, restorative therapies, and initiatives that promote equitable access to approved therapies for all people living with NMOSD.

摘要

视神经脊髓炎谱系疾病(NMOSD)是一种中枢神经系统炎症性疾病,具有独特的管理挑战。NMOSD 中的神经功能障碍与急性发作直接相关,因此,预防复发是护理的首要目标。为此,确定可预测复发发作和严重程度的有效生物标志物至关重要。随着治疗变得更加基于精准和以患者为中心,临床医生将需要熟悉 NMOSD 患者的特殊易损情况的管理,包括感染、妊娠和产后阶段。近 20 年前发现水通道蛋白-4 免疫球蛋白 G(AQP4 IgG)自身抗体,最终将 NMOSD 确定为自身免疫性星形胶质细胞病,并推动了最近的治疗进展。针对 NMOSD 中 AQP4 IgG 阳性患者的靶向治疗药物,包括依库珠单抗、satralizumab 和 inebilizumab,已被批准使用,这些药物可能会改善预后,但也存在巨大的成本。重要的是,血清阴性患者的治疗选择仍然有限。展望未来,研究探索的领域应包括预防复发、修复疗法以及促进所有 NMOSD 患者获得批准治疗的公平机会的举措。

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