背景:特发性炎性肌病（idiopathic inflammatory myopathies,IIM）是一组临床表现多样的系统性自身免疫病。包括多发性肌炎（pM）、皮肌炎（DM）、无肌病皮肌炎（ADM）、免疫介导坏死性肌病（IMNM）、包涵体肌炎及重叠综合征。该类疾病可影响到患者日常工作效率和生活质量,对此目前国内尚无相关研究。目的:研究IIM患者生活质量和工作能力障碍情况,明确其与疾病类型、疾病负担和疾病活动度的相关性。方法:收集2012年5月至2019年4月北京协和医院风湿免疫科门诊的IIM患者130例,详细记录患者的临床表现、实验室结果、疾病活动度评分等疾病相关信息,采用EQ-5D量表及WpAI问卷评估患者健康相关生活质量及工作能力障碍,同时与疾病活动度进行相关性分析;另对香港大学深圳医院2019年4月至2019年8月风湿免疫科门诊的21例患者采用sF-36量表进行生活质量自评打分,并与健康对照组进行对比。结果:130例IIM患者来自北京协和医院风湿免疫科门诊,其中DM 79例,pM 17例,ADM 32例,IMNM 1例,重叠综合征1例。根据有无合并肺间质病变分为ILD组（57例）与非ILD组（73例）;根据是否有带薪工作分为工作组（31例）和无工作组（99例）。21例IIM患者来自香港大学深圳医院风湿免疫科门诊,健康对照组34人。采用EQ-5D量表评估患者生活质量发现:DM与pM患者之间生活质量无显著性差异。ILD组与非ILD组患者生活质量无显著性差异。EQ-5D效值和手工肌力测定（MMT）之间有显著正相关性（r=0.569,p<0.01）。EQ-5D效值和肌炎活动度之间有显著负相关性（r=0.456,p<0.01）。EQ-5D效值和肌炎活动视觉模拟评估（MYOAcT）-肌肉病变评分之间有显著负相关性（r=0.624,p<0.01）。EQ5D效值和肌炎活动视觉模拟评估（MYOAcT）-整体评分之间有显著负相关性（r=0.513,p<0.01）。EQ-5D效值和患者整体评估之间有显著负相关性（r=0.644,p<0.01）。EQ-5D效值和肌炎损伤指数（MDI）整体评分之间无相关性。工作组在职工作生产力损失（presenteeism%）13.23%,因病缺勤工作生产力损失（Absenteeism%）9.79%,总工作生产力损失（Over all work impairment%）18.44%,日常活动效率损失（Activity impairment%）13.23%。在职工作生产力损失与患者整体评估有正相关性（r=0.600,p<0.01）。总工作生产力损失与患者整体评估有正相关性（r=0.480,p<0.01）。日常活动效率损失与肌炎活动视觉模拟评估（MYOAcT）-肌肉病变评分呈正相关性（r=0.397,p<0.01）。sF-36各项得分,除外精神健康（MH）及身体疼痛（Bp）两项,余生理机能（pF）、生理职能（Rp）、健康状况（GH）、精力（vT）、社会功能（sF）、情感职能（RE）、健康变化（HT）得分疾病组均低于对照组,且具有统计学显著性差异。结论:IIM患者工作能力及生活质量均存在不同程度的下降,且与疾病活动度存在明显相关性,皮肌炎和多发性肌炎亚群之间、合并ILD组与非ILD组之间,生活质量对比均不存在统计学差异。

Background: Idiopathic inflammatory myositis (IIM) is a group of systemic autoimmune diseases with various clinical manifestations, including polymyositis, dermatomyositis, amyotrophic dermatomyositis, immune-mediated necrotizing myopathy, inclusion body myositis and overlap syndrome. IIM can affect the patients' quality of life and daily work efficiency, so far there is no related research in china.
Objective: To evaluate the quality of life and the working ability of IIM patients, and to analysis the correIatiorlship with the disease type, disease burden and the disease activity.
Methods: 130 IIM patients presented in the outpatient clinic of department of Rheuinatology, peking union Medical College Hospital from May 2012 to April 2019. The clinical mnanifestations, laboratory results, disease activity scores and other disease-related information of the patients were recorded in detail. EQ-5D and WPAI questionnaires were used to evaluate the health-related quality of life and work disability, the correlation analysis was conducted with disease activity. In addition, from April 2019 to August 2019, 21 patients in the outpatient clinic of department of Rheulilatology, Hong Kong university- shen zhen Hospital, were scored by short Form-36 (SF-36) scale and compared with healthy control group.
Results: Basic data statistics: 130 IIM patients came from the rheumatology department of PUMCH, including DM(n=79), PM (n=17), ADM (n=32), IMNM (n=1) and Overlap syndrome(n=l). According to the presence of interstitial lung disease (ILD), they were divided into ILD group (n=57 ) and non~ILD group (n=73 ).According to whether having work, they were divided into working group (n=31)and non- working group (n=99). In terms of EQ-5D seale, it was found that there was no significant difference between DM and PM subgroups.
There was no significant difference between ILD group and non- TLD group either. There was a significant positive correlation between EQ-5D value score and Manual Muscle Testing-8 (MMT-8) (r=0.569, p<0.01). There was a significant negative correlation between EQ-5D value score and myositis activity (r=-0.456, p<0.01). There was a significant negative correlation between EQ-5D value score and MYOACT-musele score (r=0.624, p<0.01). There was a significant negative correIation between EQ-5D value score and MYOACT-global score(r=0.513, p<0.01). There was a significant negative correlation between EQ-5D value score and patient global activity (r=0.644, p<0.01). There is no correlation between EQ-5D value score and MDI-global score. of the 31 patients, the presenteeisin was 13.23%, the absenteeism was 9.79%, Over all work impairment and activity impairment was 18.44%, 13.23% respectively. presenteeisln is positively correlated with patient global activity (r=0.600, p<0.01), overall work impairment is positively correlated with patient global activity (r=0.480, p<0.01), activity impairment is positively correlated with MYOACT-muscle score(r=0.397, p<0.01). with regards to the scores of SF-36, physicaI function (PF), role functioning (RP), general health (GH) , vitality (VT), social function (SF), role emotion (RE), and health transition (HT) were lower than those of the control group, While mental health (MH) and body pain (BP) were not.
Conclusions: The work ability and quality of life of IIM patients decrease with different degrees, and some of them have significant correlation with disease activity. There is no significant difference between DM and PM, neither the ILD and non-ILD groups.