63例肌炎自身抗体阳性间质性肺疾病的临床特点分析  

Clinical characteristics analysis of 63 interstitial lung disease patients with positive myositis autoantibodies

作　　者：周倩[1] 唐霜 赵开娅 李欣[1] 吴彭超 唐小葵[1] Zhou Qian;Tang Shuang;Zhao Kai-Ya;Li Xin;Wu Peng-Chao;Tang Xiao-Kui(Department of Respiratory and Critical Medicine,the First Affiliated Hospital of Chongqing Medical University,Chongqing 400016,China)

机构地区：[1]重庆医科大学附属第一医院呼吸与危重症医学科,重庆400016

出　　处：《解放军医学杂志》2021年第2期142-148,共7页Medical Journal of Chinese People's Liberation Army

基　　金：重庆市科卫联合医学科研项目(2020MSXM033)。

摘　　要：目的总结分析肌炎自身抗体阳性间质性肺疾病(ILD)的临床特点,评估肌炎自身抗体检测在ILD临床诊断中的价值。方法回顾性分析重庆医科大学附属第一医院2018年1月-2019年9月收治的63例结合肌炎自身抗体明确诊断ILD患者的病例资料,分为抗合成酶综合征相关ILD(ASS-ILD)组(n=22)、多发性肌炎/皮肌炎相关ILD(PM/DM-ILD)组(n=18)与自身免疫特征的间质性肺炎(IPAF)组(n=23),分析对比三组患者的临床特点、治疗及预后情况。结果ASS-ILD组的雷诺现象、关节症状、技工手、抗ARS抗体阳性率高于IPAF组(P<0.0167)。ASS-ILD组的雷诺现象、咳嗽、呼吸困难、首发表现(呼吸系统症状)、抗ARS抗体阳性率高于PM/DM-ILD组,而发热、肌肉症状、首发表现(皮肤/肌肉症状)阳性率低于PM/DM-ILD组(P<0.0167)。PM/DM-ILD组的肌肉症状、皮肤症状、首发表现(皮肤/肌肉症状)、抗ARS抗体、抗MDA5抗体阳性率高于IPAF组,而首发表现(呼吸系统症状)阳性率低于IPAF组(P<0.0167)。63例患者胸部影像学特征以非特异性间质性肺炎(NSIP)表现为主,肺功能检查结果以弥散障碍及限制性通气障碍为主。87.3%的患者使用激素联合免疫抑制剂治疗。81.0%的患者转归为好转或稳定,19.0%的患者转归为进展恶化或死亡,三组患者转归差异无统计学意义(P>0.05)。结论ILD患者须重视肌炎自身抗体的筛查,其结果可辅助用于ILD的诊断、分类及预后评估。Objective To analyze the clinical characteristics of myositis autoantibody detection in the diagnosis of interstitial lung disease(ILD),and explore the diagnosis and treatment of ILD with the help of clinical manifestations combined with antibody results.Methods Sixty-three patients of ILD accurately diagnosed with the help of myositis autoantibody detection were collected retrospectively and divided into three groups:anti-synthetase syndrome associated interstitial lung disease group(ASS-ILD group,n=22),polymyositis/dermatomyositis associated interstitial lung disease group(PM/DM-ILD group,n=18)and interstitial pneumonia with autoimmune features group(IPAF group,n=23).Their clinical characteristics,treatment and prognosis were analyzed and compared.Results The positive rates of Raynaud’s phenomenon,joint symptoms,mechanic’s hand and anti-ARS antibodies in ASS-ILD group were higher than those in IPAF group(P<0.0167).Compared with PM/DM-ILD group,ASS-ILD group had higher positive rates of Raynaud’s phenomenon,cough,dyspnea,respiratory system symptoms as the first manifestation,anti-ARS antibodies but lower positive rates of fever,muscle symptoms,skin/muscle symptoms as the first manifestation(P<0.0167).Compared with IPAF group,PM/DM-ILD group had higher positive rates of muscle symptoms,skin symptoms,skin/muscle symptoms as the first manifestation,anti-ARS antibodies and anti-MDA5 antibody but lower positivity rates of respiratory system symptoms as the first manifestation(P<0.0167).In this study,chest radiological findings of all patients were mainly nonspecific interstitial pneumonia(NSIP).The abnormality in pulmonary functions manifested mainly as diffusion disturbance and restrictive ventilatory dysfunction.87.3%of all patients were treated with glucocorticoid combined with immunosuppressants.After treatment,81.0%of these patients showed improvement or stability,while 19.0%showed deterioration or death.The difference in prognosis between the three groups was not statistically significant(P>0.05).Conclusi

关 键 词：肺疾病 间质性 抗合成酶综合征 多发性肌炎/皮肌炎 自身免疫特征的间质性肺炎 肌炎自身抗体 

分 类 号：R563.9[医药卫生—呼吸系统]