Interleukin-6 Immunology in Macrophage Activation Syndrome Related to Neuroimmunological Diseases

Taichi Nomura,Masaaki Niino,Toshio Odani,Ryoji Naganuma,Itaru Amino,Yusei Miyazaki,Sachiko Akimoto,Zen-Ichi Tanei, Taichi Kimura,Naoya Minami,Seiji Kikuchi

Internal medicine (Tokyo, Japan)（2024）

Department of Neurology | Department of Clinical Research | Department of Rheumatology | Department of Cancer Pathology | Department of Pathology

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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.

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【要点】：本文报告了两例巨噬细胞活化综合征（MAS）并发与白细胞介素-6（IL-6）相关的神经免疫疾病，并发现IL-6受体抗体治疗能有效缓解症状。

【方法】：通过临床病例分析，探讨了IL-6在MAS和神经免疫疾病中的病理生理作用。

【实验】：无具体实验描述，但通过两个病例研究，使用标准免疫抑制治疗无效，改用IL-6受体抗体治疗后症状得到稳定。未提及具体数据集名称。