Illés Zsolt
Pécsi Tudományegyetem, Neurológiai Klinika, Pécs.
Department of Neurology, Odense University Hospital, University of Southern Denmark, Odense, Dánia.
Ideggyogy Sz. 2021 Sep 30;74(9-10):309-321. doi: 10.18071/isz.74.0309.
Treatment and new evidences in neuromyelitis optica spectrum disorder Illés Zs, MD, PhD Ideggyogy Sz 2021;74(9-10):309-321. Neuromyelitis optica spectrum disorder (NMOSD) is associated with antibodies against AQP4 in about 80% of the cases. In about one-fourth of seronegative cases, antibodies against the MOG protein are present in the serum (MOG-antibody associated disease, MOGAD). This article discusses off-label azathioprine and mycophenolate mofetil in the treatment of NMOSD and reviews the evidence-based clinical aspects of B/plasma cell depletion, antagonization of IL-6 signaling and blocking the complement pathway. The review also summarizes basic aspects of NMOSD pregnancy focusing on treatment, and the different therapeutic approach in MOGAD. In the recent two years, phase 3 clinical trials provided class I evidence for the efficacy and safety of rituximab (anti-CD20), inebilizumab (anti-CD19), tocilizumab (anti-IL6R), satralizumab (anti-IL6R), and eculizumab (anti-C5) in combination with other immunosuppressants or in monotherapy. The treatment approach in MOGAD is complicated by the monophasic course in about half of the cases and by the potential disappearance of MOG antibody. The necessity of maintenance treatment in MOGAD should be decided after tapered oral steroid. Immunosuppression is recommended in NMOSD during pregnancy and lactation, and this should be considered for optimal selection of treatment in fertile female patients. The new monoclonal antibodies broadened treatment options NMOSD, and the treatment strategy of MOGAD has become more straightforward.
视神经脊髓炎谱系障碍的治疗与新证据 伊莱斯·兹斯，医学博士、哲学博士 《Ideggyogy Sz》2021年；74(9 - 10):309 - 321。约80%的视神经脊髓炎谱系障碍（NMOSD）病例与抗水通道蛋白4抗体相关。在约四分之一的血清阴性病例中，血清中存在抗髓鞘少突胶质细胞糖蛋白（MOG）抗体（MOG抗体相关疾病，MOGAD）。本文讨论了硫唑嘌呤和霉酚酸酯在NMOSD治疗中的超说明书用药情况，并综述了B细胞/浆细胞清除、IL - 6信号拮抗和补体途径阻断的循证临床方面。该综述还总结了NMOSD妊娠的基本方面，重点是治疗以及MOGAD的不同治疗方法。在最近两年中，3期临床试验为利妥昔单抗（抗CD20）、依奈利珠单抗（抗CD19）、托珠单抗（抗IL - 6R）、萨特利珠单抗（抗IL - 6R）和依库珠单抗（抗C5）与其他免疫抑制剂联合使用或单药治疗的疗效和安全性提供了I类证据。MOGAD的治疗方法因约一半病例的单相病程以及MOG抗体可能消失而变得复杂。MOGAD维持治疗的必要性应在口服类固醇减量后决定。NMOSD患者在妊娠和哺乳期建议进行免疫抑制，对于育龄期女性患者的最佳治疗选择应考虑这一点。新的单克隆抗体拓宽了NMOSD的治疗选择，MOGAD的治疗策略也变得更加直接。
