Tomimitsu Hiroyuki
Department of Neurology, JA Toride Medical Center.
Brain Nerve. 2021 Feb;73(2):127-136. doi: 10.11477/mf.1416201725.
Immune-mediated necrotizing myopathy (IMNM) is a heterogeneous disease that presents with subacute progressive proximal dominant muscle weakness clinically and prominent necrotic muscle fibers without invasion of inflammatory cells pathologically. IMNM is a disease that exclusively affects skeletal muscles, so the serum creatine kinase level usually increases prominently. It is thought to be an autoimmune disease because of the presence of two autoantibodies, anti-SRP and anti-HMGCR. Because muscle involvement is more severe than that in other forms of myositis, its functional prognosis is worse. Several immune-modifying therapies can improve the symptoms; therefore, intensive treatments should be started soon after diagnosis with IMNM.
免疫介导的坏死性肌病(IMNM)是一种异质性疾病,临床上表现为亚急性进行性近端为主的肌无力,病理上可见明显的坏死肌纤维,而无炎性细胞浸润。IMNM是一种仅累及骨骼肌的疾病,因此血清肌酸激酶水平通常显著升高。由于存在两种自身抗体,即抗信号识别颗粒(SRP)抗体和抗3-羟基-3-甲基戊二酰辅酶A还原酶(HMGCR)抗体,故认为它是一种自身免疫性疾病。由于其肌肉受累程度比其他类型的肌炎更严重,其功能预后较差。几种免疫调节疗法可改善症状;因此,确诊IMNM后应尽早开始强化治疗。