特发性炎性肌病不同亚型临床特征、肌炎抗体谱、肌电图的差异性表现  

Differential clinical characteristics, myositis antibody spectrum, and electromyography of different subtypes of idiopathic inflammatory myopathy

作　　者：李筱筱 李雨薇 孙莉 马艳 Li Xiaoxiao;Li Yuwei;Sun Li;Ma Yan(Department of Rheumatology and Immunology,the First Affiliated Hospital of University of Science and Technology of China,Anhui Province,Hefei 230000,China)

机构地区：[1]中国科技大学附属第一医院风湿免疫科,合肥230000

出　　处：《疑难病杂志》2023年第5期515-519,共5页Chinese Journal of Difficult and Complicated Cases

基　　金：安徽省教育厅计划项目(2019jyxm0950)。

摘　　要：目的研究特发性炎性肌病(IIM)患者不同亚型间临床特征、肌炎抗体谱、肌电图的差异。方法选取2017年2月—2021年2月于中国科技大学附属第一医院风湿免疫科收治的IIM患者179例临床资料,并根据不同类型分为皮肌炎(DM)组135例,多肌炎(PM)组26例,无肌病性皮肌炎(CADM)组18例,检测患者血清肌炎特异性抗体(MSAs)水平及肌电图,比较3组IIM患者的临床特征、肌炎抗体谱、肌电图结果。结果179例IIM患者中,DM、PM、CADM 3种类型中患者均表现出肌肉疼痛,但CADM患者则较少出现肌无力的表现;DM、CADM患者几乎均存在经典的皮肤表现(向阳疹、披肩征、V字征、Gottron征),PM患者基本无皮肤表现;在DM、CADM患者中,向阳疹、披肩征、V字征的频率相近,CADM组Gottron征出现频率高于DM组(P<0.05)。12种MSAs中,抗MDA5抗体(35.2%)最常见,其次是抗ARS抗体(34.1%,其中抗Jo-1抗体阳性率最高)、抗TIF1γ抗体(10.6%)和抗Mi2β抗体(8.3%);抗MDA5抗体阳性率比较,CADM组>DM组>PM组(χ^(2)=19.220,P<0.001);CADM组患者无抗Jo-1抗体、抗SRP抗体,PM组患者抗Jo-1抗体、抗SRP抗体阳性率高于DM组(P<0.05),CADM组患者抗OJ抗体阳性率高于PM组和DM组(P<0.05);余抗体在3组患者中的表达差异无统计学意义(P>0.05)。179例患者中112例送检肌电图,105例(93.8%)肌电图结果提示存在异常,肌源性损害88例(78.6%),神经源性损害54例(48.2%)。DM、PM、CADM组之间肌电图异常率、肌源性损害、神经传导速度异常比较差异无统计学意义(P>0.05)。结论DM、PM、CADM 3种亚型的IIM患者可出现不同程度的肌痛,CADM患者以皮肤病变为主,较少出现肌无力的表现。抗MDA5抗体是最常见的MSAs,其次是抗ARS抗体。不同肌炎类型患者的肌电图均存在肌源性损害,部分受损肌肉可表现神经源性损害。Objective To investigate the differences in clinical characteristics,myositis antibody spectrum,and electromyography among different subtypes of idiopathic inflammatory myopathy(IIM)patients.Methods One hundred and seventy-nine IIM patients admitted to the Rheumatology and Immunology Department of the First Affiliated Hospital of the University of Science and Technology of China from February 2017 to February 2021 were selected for clinical data.They were divided into a dermatomyositis(DM)group of 135 cases,a polymyositis(PM)group of 26 cases,and a non-myositis dermatomyositis(CADM)group of 18 cases based on different types.The serum levels of myositis specific antibodies(MSAs)and electromyography of the patients were measured,and the clinical characteristics,myositis antibody spectrum,and electromyographic results of the three groups of IIM patients were compared.Results Among 179 IIM patients,DM,PM,and CADM all showed muscle pain,but CADM patients did not show muscle weakness;Almost all patients with DM and CADM have classic skin manifestations(periorbital edema,shawl sign,V-zone rash,high snow sign),while PM patients have no skin manifestations;In DM and CADM patients,the frequency of periorbital edema,shawl sign,and V-zone rash is similar,while the frequency of high snow sign in the CADM group is higher than that in the DM group(P<0.05).Among the 12 types of MSAs,anti MDA5 antibodies(35.2%)are the most common,followed by anti ARS antibodies(34.1%,with the highest positive rate of anti Jo-1 antibodies)and anti TIF1γAntibodies(10.6%)and anti Mi2βAntibodies(8.3%);Comparison of positive rates of anti-MDA5 antibodies in the CADM group>DM group>PM group(χ^(2)=19.220,P<0.001);The CADM group had no anti Jo-1 or anti SRP antibodies,while the positive rates of anti Jo-1 and anti SRP antibodies in the PM group were higher than those in the DM group(P<0.05).The positive rates of anti OJ antibodies in the CADM group were higher than those in the PM and DM groups(P<0.05);There was no statistically significant difference

关 键 词：特发性炎性肌病 皮肌炎 多肌炎 无肌病性皮肌炎 肌电图 

分 类 号：R[医药卫生]