Instituto de Investigación en Reumatología y del Sistema Músculo Esqueletico, Departamento de Biología Molecular, Centro Universitario de Ciencias de la Salud, Universidad de Guadalajara, Guadalajara, Mexico.
Departamento de Fisiología, Centro Universitario de Ciencias de la Salud, Universidad de Guadalajara, Guadalajara, Mexico.
Front Immunol. 2022 Sep 15;13:940122. doi: 10.3389/fimmu.2022.940122. eCollection 2022.
Idiopathic inflammatory myopathies (IIMs) are a group of rare, acquired autoimmune diseases characterized by profound muscle weakness and immune cell invasion into non-necrotic muscle. They are related to the presence of antibodies known as myositis-specific antibodies and myositis-associated antibodies, which are associated with various IIM phenotypes and the clinical prognosis. The possibility of the participation of other pathological mechanisms involved in the inflammatory response in IIM has been proposed. Such mechanisms include the overexpression of major histocompatibility complex class I in myofibers, which correlates with the activation of stress responses of the endoplasmic reticulum (ER). Taking into account the importance of the ER for the maintenance of homeostasis of the musculoskeletal system in the regulation of proteins, there is probably a relationship between immunological and non-immunological processes and autoimmunity, and an example of this might be IIM. We propose that ER stress and its relief mechanisms could be related to inflammatory mechanisms triggering a humoral response in IIM, suggesting that ER stress might be related to the triggering of IIMs and their auto-antibodies' production.
特发性炎性肌病（IIM）是一组罕见的获得性自身免疫性疾病，其特征为严重的肌肉无力和免疫细胞浸润非坏死性肌肉。它们与被称为肌炎特异性抗体和肌炎相关抗体的抗体有关，这些抗体与各种 IIM 表型和临床预后有关。已经提出了其他涉及肌炎炎症反应的病理机制参与的可能性。这些机制包括肌纤维中主要组织相容性复合体 I 的过度表达，这与内质网（ER）应激反应的激活相关。考虑到 ER 在调节蛋白质的肌肉骨骼系统的动态平衡中的重要性，免疫和非免疫过程与自身免疫之间可能存在关系，其中一个例子可能是 IIM。我们提出 ER 应激及其缓解机制可能与触发 IIM 体液反应的炎症机制有关，这表明 ER 应激可能与 IIM 的触发及其自身抗体的产生有关。
