摘要
免疫介导坏死性肌病(immune-mediated necrotizing myopathy,IMNM)是一种特发性炎性肌病(Idio-pathic Inflammatory Myopathy,IIM)的重要亚型.IMNM的典型临床表现包括对称性四肢近端肌无力,伴随血清肌酸激酶(creatine kinase,CK)等肌酶谱的显著升高.此外,部分患者还可能出现皮疹、间质性肺病和心肌受累等骨骼肌外表现.自2003年首次被国际医学界命名以来,IMNM逐渐被研究者和临床医师所认识.IMNM的发病率和患病率在不同地域和种族间可能有所不同,如在美国的小样本调查中,IMNM的发病率为0.83/10万,患病率为1.85/10万;而在欧洲西班牙北部地区,抗HMGCR抗体阳性患者的IMNM发病率为0.6/10万,患病率为3/10万,目前中国尚缺乏相关数据.IMNM的患病风险因素包括易感等位基因如HLA-DRB1*11,以及使用他汀类药物、免疫检查点抑制剂和感染病毒感染等.诊断IMNM需要综合考虑肌肉受累症状、肌炎特异性自身抗体、肌酶谱水平和肌活检病理等检查结果.目前,IMNM的治疗主要基于临床经验,包括使用糖皮质激素和传统免疫抑制剂.对于难治性患者,可能采用利妥昔单抗和静脉注射人免疫球蛋白等治疗方案.针对B细胞及其致病性自身抗体的产生过程,未来的研究可能揭示新的治疗靶点.本文旨在系统介绍IMNM的临床特征、诊断方法及相关辅助检查,并探讨当前的治疗策略,以期为临床医师提供诊治IMNM的参考和借鉴.
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.
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