Abstract:
Immune-mediated necrotizing myopathy (IMNM) is a class of idiopathic inflammatory myopathies. According to the types of antibodies in serum, IMNM can be divided into three subtypes: anti-signal recognition particle (SRP) necrotizing myopathy, 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) necrotizing myopathy, and serum antibody-negative necrotizing myopathy. Different subtypes of IMNM have common characteristics, but the specific pathological mechanisms differ. Anti-SRP necrotizing myopathy is an important type of IMNM. At present, the pathogenesis of the disease is unclear. Most views suggest that the disease is mainly caused by an autoimmune response; therefore, the therapeutic strategy is primarily immune regulation. Recent studies have implicated non-immune mechanisms such as endoplasmic reticulum stress and autophagy in the occurrence and development of the disease. Here, we review what is known about the pathogenesis of anti-SRP necrotizing myopathy and summarize the latest research progress, aiming to better understand the disease and provide new ideas for treatment targets.
摘要:
免疫介导的坏死性肌病(IMNM)是一类特发性炎性肌病。根据血清中抗体的类型,IMNM可分为三种亚型:抗信号识别粒子(SRP)坏死性肌病,3-羟基-3-甲基戊二酰辅酶A还原酶(HMGCR)坏死性肌病,血清抗体阴性坏死性肌病。IMNM的不同亚型具有共同的特征,但具体的病理机制不同。抗SRP坏死性肌病是IMNM的重要类型。目前,该病的发病机制尚不清楚。大多数观点认为该疾病主要是由自身免疫反应引起的;因此,治疗策略主要是免疫调节。近年来研究发现内质网应激、自噬等非免疫机制参与了该病的发生发展。这里,本文综述了抗SRP坏死性肌病发病机制的研究进展,旨在更好地了解该病,为治疗靶点提供新的思路。