摘要
Abstract
Immune-mediated necrotizing myopathy(IMNM)is a significant,subtype of idiopathic inflammatory my-opathy(IIM),characterized by symmetrical proximal limb muscle weakness and markedly elevated serum creatine kinase levels.Some patients may also exhibit extra-skeletal muscle manifestations,including rashes,interstitial lung disease,and myocardial involvement.Since its international nomenclature in 2003,IMNM has gained increasing recognition among re-searchers and clinicians,leading to numerous scientific investigations and clinical applications.The incidence and preva-lence of IMNM can vary across different geographic regions and ethnic groups.A small sample survey conducted in the United States reported an incidence of 0.83 per 100,000 and a prevalence of 1.85 per 100,000.In northern Spain,among patients positive for anti-HMGCR antibodies,the incidence was found to be 0.6 per 100,000,with a prevalence of 3 per 100,000.However,there is a notable paucity of relevant data regarding IMNM in China.Identified risk factors for IMNM in-clude susceptibility alleles(e.g.,HLA-DRB1*11),the use of statins or immune checkpoint inhibitors,and viral infections.Diagnosing IMNM requires a comprehensive evaluation that includes assessing muscle involvement symptoms,identifying myositis-specific autoantibodies,measuring creatine kinase levels,analyzing muscle biopsy pathology,and conducting re-lated examinations.Currently,there is a lack of prospective randomized controlled studies on the treatment of IMNM.In clinical practice,glucocorticoids and traditional immunosuppressants are primarily employed on an empirical basis.For re-fractory cases,treatment regimens may involve rituximab and intravenous human immunoglobulin.Therapeutic strategies targeting B cells and the mechanisms underlying pathogenic autoantibody production may offer promising avenues for fu-ture treatment.This article systematically reviews the clinical characteristics,diagnostic criteria,relevant auxiliary exami-nations,and treatment strategies for IMNM,aiming to provide a comprehensive reference for clinicians in understanding,di-agnosing,and managing this condition.关键词
免疫介导坏死性肌病/肌炎特异性抗体/靶向治疗Key words
Immune mediated necrotizing myopathy/Myositis specific antibodies/Targeted therapy分类
医药卫生
