The final, formatted version of the article will be published soon.
CASE REPORT article
Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders
Volume 15 - 2024 |
doi: 10.3389/fimmu.2024.1528989
This article is part of the Research Topic Advances in Antigen-Specific Immunotherapies for Autoimmune Disease Management View all 8 articles
Inebilizumab Treatment in a Patient with Co-occurring AQP4-IgG Positive Neuromyelitis Optica Spectrum Disorder and Myasthenia Gravis: A Case Report
Provisionally accepted- 1 The Affiliated Hospital of Qingdao University, Qingdao, China
- 2 Qingdao University, Qingdao, Shandong Province, China
Objective: This study aims to delineate the clinical features underlying the concurrent disease of neuromyelitis optica spectrum disorder (NMOSD) and myasthenia gravis (MG), and to identify efficacious therapeutic strategies.Background: NMOSD and MG are uncommon autoimmune diseases that infrequently co-exist. Despite previous reports, a consensus on treating NMOSD concurrent with MG is lacking.We present the case of a 55-year-old female with both anti-aquaporin-4 (AQP4) antibody-positive NMOSD and anti-acetylcholine receptor (AChR) antibody-positive MG, who achieved stable disease control following treatment with inebilizumab without significant adverse effects. We also conducted a literature review to evaluate the clinical profile of this comorbidity.Results: Our review identified 85 patients with concurrent NMOSD and MG. In 70 well-documented cases, MG predated NMOSD in 60 (85.8%) cases, with 42 (70%) patients having undergone thymectomy. Six (8.6%) patients were first diagnosed with NMOSD, and then thymectomy was performed in 2 (33.3%) MG patients. For NMOSD treatment, although most patients received steroid hormones and immunosuppressive agents, quite a few patients had persistent severe disability.Additionally, of 44 patients with clear records of disease recurrence, 31 patients(70.5%) experienced frequent relapses of optic neuritis and myelitis, ranging from 1 to 15 attacks, averaging five. The manifestations of MG are mainly included fatigability, diplopia, and blepharoptosis, with symptoms well-controlled in most patients. Our patient treated with inebilizumab for 1 year and no relapse was recorded to date.Though MG typically precedes NMOSD and thymectomy is frequently performed, it is not a prerequisite for NMOSD development but may represent a potential risk factor. MG generally follows a benign course, in contrast to the more aggressive nature of NMOSD. The utility of biological agents such as inebilizumab for patients with both NMOSD combined with MG warrants further attention.
Keywords: Neuromyelitis optica spectrum disorder, Myasthenia Gravis, Inebilizumab, anti-AQP4 antibodies, anti-acetylcholine receptors antibodies
Received: 15 Nov 2024; Accepted: 18 Dec 2024.
Copyright: © 2024 Song, Chen, Jin, Peng, Sun and Zheng. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence:
Xueping Zheng, The Affiliated Hospital of Qingdao University, Qingdao, China
Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.