免疫介导的坏死性肌病(IMNM)是特发性炎性肌病范围内的一种罕见且新近识别的自身免疫性疾病。它的特点是肌炎特异性自身抗体,血清肌酸激酶水平升高,炎症浸润,和弱点。根据特异性自身抗体的存在或不存在,IMNM可以分为三种亚型:抗信号识别颗粒肌炎,抗3-羟基-3-甲基戊二酰辅酶A还原酶肌炎,和血清阴性IMNM。近年来,IMNM越来越受到关注,成为研究热点。最近的研究表明,IMNM的发病机制与免疫系统的异常激活有关。包括抗体介导的免疫反应,补语,和免疫细胞,特别是巨噬细胞,以及炎症因子的异常释放。自噬和内质网应激等非免疫机制也参与了这一过程。此外,已经确定了与IMNM相关的遗传变异,提供对疾病遗传机制的新见解。IMNM治疗研究也取得了进展,包括免疫抑制剂的使用和生物制剂的开发。尽管在理解IMNM的病因和治疗方面存在挑战,最新的研究结果为深入研究该疾病的致病机制和确定新的治疗策略提供了重要的指导和见解。
Immune-mediated necrotizing myopathy (IMNM) is a rare and newly recognized autoimmune disease within the spectrum of idiopathic inflammatory myopathies. It is characterized by myositis-specific autoantibodies, elevated serum creatine kinase levels, inflammatory infiltrate, and weakness. IMNM can be classified into three subtypes based on the presence or absence of specific autoantibodies: anti-signal recognition particle myositis, anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase myositis, and seronegative IMNM. In recent years, IMNM has gained increasing attention and emerged as a research hotspot. Recent studies have suggested that the pathogenesis of IMNM is linked to aberrant activation of immune system, including immune responses mediated by antibodies, complement, and immune cells, particularly macrophages, as well as abnormal release of inflammatory factors. Non-immune mechanisms such as autophagy and endoplasmic reticulum stress also participate in this process. Additionally, genetic variations associated with IMNM have been identified, providing new insights into the genetic mechanisms of the disease. Progress has also been made in IMNM treatment research, including the use of immunosuppressants and the development of biologics. Despite the challenges in understanding the etiology and treatment of IMNM, the latest research findings offer important guidance and insights for delving deeper into the disease\'s pathogenic mechanisms and identifying new therapeutic strategies.