Successful treatment with efgartigimod as an add-on therapy in acute attack of anti-AQP4 antibody-positive NMOSD: a case report.

IF 2.7 4区医学 Q2 CLINICAL NEUROLOGY Neurological Sciences Pub Date : 2024-11-01 Epub Date: 2024-07-06 DOI:10.1007/s10072-024-07678-3

Shi-Qi Huang, Zhen-Hua Yuan, Ye Hong, Teng Jiang, Hong-Dong Zhao, Jian-Quan Shi

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Abstract

Background: Neuromyelitis Optica Spectrum Disorder (NMOSD) is an autoimmune demyelinating disease characterized by recurrent myelitis and optic neuritis. It is associated with high rates of relapse and disability. The main treatment strategies for acute attacks include intravenous methylprednisolone pulse (IVMP) treatment and rescue treatment with plasma exchange (PLEX). Recently, the blockade of neonatal Fc receptor (FcRn)-IgG interaction has gained momentum as a therapeutic strategy. Efgartigimod, the first approved FcRn inhibitor for treating generalized myasthenia gravis, has shown impressive safety, efficacy, and tolerability, and is being regarded as "PLEX in a bottle".

Case description: We report a 65-year-old female patient who was diagnosed with anti-AQP4 antibody positive NMOSD. Add-on treatment with efgartigimod to IVMP and intravenous immunoglobulin (IVIG) at the second acute relapse showed favorable results.

Conclusion: This case suggests that efgartigimod is a potentially effective add-on therapy in acute attacks of AQP4-IgG-positive NMOSD.

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在抗AQP4抗体阳性NMOSD急性发作期使用依加替莫德作为附加疗法取得成功：病例报告。

背景：神经脊髓炎谱系障碍（NMOSD）是一种自身免疫性脱髓鞘疾病，以反复发作的脊髓炎和视神经炎为特征。这种疾病的复发率和致残率都很高。急性发作期的主要治疗策略包括静脉甲基强的松龙脉冲（IVMP）治疗和血浆置换（PLEX）抢救治疗。最近，阻断新生儿 Fc 受体（FcRn）-IgG 相互作用已成为一种治疗策略。埃加替莫德是首个获批用于治疗全身性肌无力的 FcRn 抑制剂，其安全性、有效性和耐受性令人印象深刻，被誉为 "瓶中的 PLEX"：我们报告了一名 65 岁的女性患者，她被诊断为抗 AQP4 抗体阳性的 NMOSD。在第二次急性复发时，在IVMP和静脉注射免疫球蛋白（IVIG）的基础上加用依加替莫德治疗，结果显示良好：本病例表明，依加替莫德是治疗AQP4-IgG阳性NMOSD急性发作的一种潜在有效的附加疗法。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Neurological Sciences 医学-临床神经学

CiteScore

6.10

自引率

3.00%

发文量

743

审稿时长

4 months

期刊介绍： Neurological Sciences is intended to provide a medium for the communication of results and ideas in the field of neuroscience. The journal welcomes contributions in both the basic and clinical aspects of the neurosciences. The official language of the journal is English. Reports are published in the form of original articles, short communications, editorials, reviews and letters to the editor. Original articles present the results of experimental or clinical studies in the neurosciences, while short communications are succinct reports permitting the rapid publication of novel results. Original contributions may be submitted for the special sections History of Neurology, Health Care and Neurological Digressions - a forum for cultural topics related to the neurosciences. The journal also publishes correspondence book reviews, meeting reports and announcements.