肌炎特异性抗体阳性的多发性肌炎/皮肌炎相关间质性肺疾病22例临床特点分析

Analysis of clinical characteristics of 22 cases of polymyositis/dermatomyositis-related interstitial lung disease with positive myositis-specific antibodies

目的总结分析多发性肌炎/皮肌炎(PM/DM)相关间质性肺疾病(Interstitial Lung Diseases,ILD)的临床特点,探讨ILD诊断中肌炎抗体检测的重要性。方法回顾性分析安徽省胸科医院2019年4月-2021年6月收治的22例肌炎特异抗体阳性的PM/DM-ILD患者的病例资料,根据临床特征分为抗合成酶综合征(anti-synthetase syndrome,ASS)相关间质性肺疾病组(ASS-ILD)(13例)、抗黑色素瘤分化相关基因5(Melanoma differentiation-associated gene 5,MDA5)阳性相关间质性肺疾病组(MDA5-ILD)(5例)、其他PM/DM相关间质性肺疾病组(4例),对比三组患者临床特点、治疗及预后。结果ASS-ILD组易出现技工手(11例,84.6%)及雷诺现象(9例,69.2%),MDA5-ILD组肌无力症状(5例,100%)、Gottron征(4例,80.0%)及眶周紫斑(3例,60.0%)发生率较高,3组之间比较有统计学意义(P<0.05)。其他PM/DM相关ILD组肌酸激酶(CK)升高更明显(1例,25.0%)。22例患者胸部影像学特征以机化性肺炎(Organizing pneumonia,OP)最为多见(11例,50.0%)。转归方面,MDA5-ILD组死亡率高(4例,80.0%),ASS-ILD组最低(1例,7.7%)。结论肌炎抗体筛查对ILD的诊治及预后评估具有重要意义。

Objective To summarize and analyze the clinical characteristics of Interstitial Lung Diseases(ILD)-related polymyositis/dermatomyositis(PM/DM),and to explore the importance of myositis antibody detection in the diagnosis of ILD.Methods The clinical data of 22 PM/DM-ILD patients with positive myositis specific antibodies who were admitted to Anhui Chest Hospital from April 2019 to June 2021 were retrospectively analyzed.According to clinical characteristics,they were divided into anti-synthetase syndrome(ASS)-related interstitial lung disease(ASS-ILD)group(13 cases),anti-melanoma differentiation-associated gene 5(MDA5)positive related interstitial lung disease(MDA5-ILD)group(5 cases),and other PM/DM related interstitial lung disease group(4 cases).The clinical characteristics,treatment,and prognosis of the three groups were compared.Results Artisan hand(11 cases,84.6%)and Raynaud′s phenomenon(9 cases,69.2%)were more common in the ASS-ILD group,and the incidence of muscle weakness(5 cases,100%),Gottron′s sign(4 cases,80.0%),and periorbital viola(3 cases,60.0%)were higher in MDA5-ILD group.There was statistical significance among the three groups(P<0.05).Creatine kinase(CK)was increased significantly in other PM/DM-related ILD groups(1 case,25.0%).The most common chest imaging feature of 22 patients was Organizing pneumonia(OP)(11 cases,50.0%).In terms of outcome,the mortality rate in the MDA5-ILD group was high(4 cases,80.0%),and the ASS-ILD group was the lowest(1 case,7.7%).Conclusion Myositis antibody screening is of great significance for the diagnosis,treatment,and prognosis of ILD.