{"title":"Interleukin-6 Immunology in Macrophage Activation Syndrome Related to Neuroimmunological Diseases.","authors":"Taichi Nomura, Masaaki Niino, Toshio Odani, Ryoji Naganuma, Itaru Amino, Yusei Miyazaki, Sachiko Akimoto, Zen-Ichi Tanei, Taichi Kimura, Naoya Minami, Seiji Kikuchi","doi":"10.2169/internalmedicine.3757-24","DOIUrl":null,"url":null,"abstract":"<p><p>Macrophage activation syndrome (MAS) involves an excessive amount of acute inflammatory responses to inflammatory cytokines, particularly interleukin-6 (IL-6). IL-6 is also strongly associated with the pathophysiology of certain neuroimmunological diseases. However, there have so far been few reports of MAS being accompanied by neuroimmunological diseases. We herein report two cases of MAS comorbid with myasthenia gravis or neuromyelitis optica spectrum disorders, IL-6 related neuroimmunological diseases. Standard immunosuppressive therapies could not stabilize the symptoms in our cases until antibodies against the IL-6 receptor were administered. This finding suggests that it is important to consider the underlying pathophysiology of MAS in relation to these neuroimmunological diseases when treating affected patients.</p>","PeriodicalId":13719,"journal":{"name":"Internal Medicine","volume":" ","pages":"941-945"},"PeriodicalIF":1.0000,"publicationDate":"2025-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Internal Medicine","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.2169/internalmedicine.3757-24","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/8/28 0:00:00","PubModel":"Epub","JCR":"Q3","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}
引用次数: 0
Abstract
Macrophage activation syndrome (MAS) involves an excessive amount of acute inflammatory responses to inflammatory cytokines, particularly interleukin-6 (IL-6). IL-6 is also strongly associated with the pathophysiology of certain neuroimmunological diseases. However, there have so far been few reports of MAS being accompanied by neuroimmunological diseases. We herein report two cases of MAS comorbid with myasthenia gravis or neuromyelitis optica spectrum disorders, IL-6 related neuroimmunological diseases. Standard immunosuppressive therapies could not stabilize the symptoms in our cases until antibodies against the IL-6 receptor were administered. This finding suggests that it is important to consider the underlying pathophysiology of MAS in relation to these neuroimmunological diseases when treating affected patients.
巨噬细胞活化综合征(MAS)涉及对炎症细胞因子,尤其是白细胞介素-6(IL-6)的过度急性炎症反应。IL-6 也与某些神经免疫疾病的病理生理学密切相关。然而,迄今为止,有关 MAS 同时伴有神经免疫疾病的报道还很少。我们在此报告了两例 MAS 合并重症肌无力或神经脊髓炎谱系障碍(IL-6 相关神经免疫疾病)的病例。在使用抗 IL-6 受体的抗体之前,标准的免疫抑制疗法无法稳定病例的症状。这一发现表明,在治疗受影响的患者时,必须考虑到MAS的潜在病理生理学与这些神经免疫疾病的关系。
期刊介绍:
Internal Medicine is an open-access online only journal published monthly by the Japanese Society of Internal Medicine.
Articles must be prepared in accordance with "The Uniform Requirements for Manuscripts Submitted to Biomedical Journals (see Annals of Internal Medicine 108: 258-265, 1988), must be contributed solely to the Internal Medicine, and become the property of the Japanese Society of Internal Medicine. Statements contained therein are the responsibility of the author(s). The Society reserves copyright and renewal on all published material and such material may not be reproduced in any form without the written permission of the Society.