Liang Junyu, Cao Heng, Ke Yini, Sun Chuanyin, Chen Weiqian, Lin Jin
Department of Rheumatology, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, China.
Front Med (Lausanne). 2020 Jan 31;7:12. doi: 10.3389/fmed.2020.00012. eCollection 2020.
This study aimed at clarifying the prevalence, risk factors, outcome, and outcome-related factors of acute exacerbation of interstitial lung disease (AE-ILD) in patients with idiopathic inflammatory myopathy (IIM). Data of IIM patients who were admitted to the First Affiliated Hospital of Zhejiang University (FAHZJU) from September 2007 to September 2019 were retrospectively collected. And the IIM patients with AE-ILD formed the case group. In addition, age and sex matched IIM patients without AE-ILD were randomly selected to constitute the control group. A 1:2 case-control study and intragroup analysis were performed to identify risk factors for development of AE-ILD in IIM patients and unfavorable short-term outcome in AE-ILD patients through comparison, univariate and multivariate logistic regression analysis. AE-ILD occurred in 64 out of 665 IIM patients (9.6%) with a short-term mortality rate of 39.1%. And the 64 IIM patients with AE-ILD formed the case group. Besides, 128 age and sex matched IIM patients without AE-ILD were randomly selected to constitute the control group. The retrospective case-control study revealed that elevated on-admission disease activity ( < 0.001), lower percent-predicted diffusing capacity of the lung for carbon monoxide (DLCO%, = 0.013) and diagnosis of clinically amyopathic dermatomyositis (CADM, = 0.007) were risk factors for development of AE-ILD in IIM patients. The following intragroup analysis indicated that elevated on-admission disease activity ( = 0.008) and bacterial infection ( = 0.003) were significantly correlated with the unfavorable short-term outcome of patients complicated with AE-ILD. In addition, combined use of steroid and disease modifying antirheumatic drugs (DMARDs, = 0.006) was found to significantly reduce the short-term mortality in IIM patients with AE-ILD. AE-ILD is a less frequent but fatal complication in IIM patients with elevated on-admission disease activity, lower DLCO% and diagnosis of CADM working as risk factors, indicating the potential roles of autoimmune abnormality and hypoxia in development of AE-ILD. Elevated on-admission disease activity and bacterial infection could predict unfavorable short-term outcome of IIM patients with AE-ILD. A therapeutic regimen of steroid and DMARDs was found to reduce short-term death in these patients.
本研究旨在阐明特发性炎性肌病（IIM）患者间质性肺病急性加重（AE-ILD）的患病率、危险因素、结局及与结局相关的因素。回顾性收集了2007年9月至2019年9月在浙江大学医学院附属第一医院（FAHZJU）住院的IIM患者的数据。其中发生AE-ILD的IIM患者组成病例组。此外，随机选取年龄和性别匹配的无AE-ILD的IIM患者组成对照组。进行1:2病例对照研究和组内分析，通过比较、单因素和多因素逻辑回归分析，确定IIM患者发生AE-ILD的危险因素以及AE-ILD患者短期不良结局的危险因素。665例IIM患者中有64例（9.6%）发生AE-ILD，短期死亡率为39.1%。这64例发生AE-ILD的IIM患者组成病例组。另外，随机选取128例年龄和性别匹配的无AE-ILD的IIM患者组成对照组。回顾性病例对照研究显示，入院时疾病活动度升高（<0.001）、肺一氧化碳弥散量预测值百分比降低（DLCO%，=0.013）以及临床无肌病性皮肌炎（CADM）诊断（=0.007）是IIM患者发生AE-ILD的危险因素。随后的组内分析表明，入院时疾病活动度升高（=0.008）和细菌感染（=0.003）与合并AE-ILD患者的短期不良结局显著相关。此外，发现联合使用类固醇和改善病情抗风湿药物（DMARDs，=0.006）可显著降低发生AE-ILD的IIM患者的短期死亡率。AE-ILD在入院时疾病活动度升高、DLCO%降低及CADM诊断的IIM患者中是一种较少见但致命的并发症，提示自身免疫异常和缺氧在AE-ILD发生过程中的潜在作用。入院时疾病活动度升高和细菌感染可预测合并AE-ILD的IIM患者的短期不良结局。发现类固醇和DMARDs的治疗方案可降低这些患者的短期死亡率。
