Neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorder (NMOSD) are autoimmune diseases associated with a disease-specific autoantibody directed against the water channel protein aquaporin-4. Standard immunotherapy, immunosuppressive agents, and corticosteroids can prevent acute attacks and maintain remission in most patients with NMOSD. However, there is a strong need for additional options for patients who are refractory to standard treatments. Emerging therapies targeting specific molecules related to the pathogenicity of NMOSD are currently being developed. The review focuses on improving preventive treatments for NMOSD, including ongoing randomized clinical trials using biological drugs targeting CD19 and CD20 on B cells, interleukin-6, and complement protein C5. The anti-IL-6 receptor monoclonal antibody tocilizumab (TCZ), which can block IL-6 signaling, was shown to be highly effective for refractory patients with NMOSD. Notably, TCZ has marked effects on chronic neuropathic pain and general fatigue in patients refractory to standard medications. TCZ is a promising drug for preventing acute attacks in patients with NMOSD.

中文翻译：

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IL-6信号在视神经脊髓炎中的阻断作用。

视神经脊髓炎（NMO）和视神经脊髓光谱症（NMOSD）是与针对水通道蛋白aquaporin-4的疾病特异性自身抗体相关的自身免疫性疾病。标准的免疫疗法，免疫抑制剂和皮质类固醇可以预防大多数NMOSD患者的急性发作并维持缓解。但是，对于标准治疗难以耐受的患者，强烈需要其他选择。目前正在开发针对与NMOSD致病性相关的特定分子的新兴疗法。综述的重点是改善NMOSD的预防性治疗，包括正在进行的针对B细胞，白介素6和补体蛋白C5靶向CD19和CD20的生物药物的随机临床试验。抗IL-6受体单克隆抗体托珠单抗（TCZ）可以阻断IL-6信号转导的NMOSD对难治性NMOSD患者非常有效。值得注意的是，TCZ对难于使用标准药物治疗的患者的慢性神经性疼痛和全身疲劳有显着影响。TCZ是一种用于预防NMOSD患者急性发作的有前途的药物。