Table_1_Predictors and Mortality of Rapidly Progressive Interstitial Lung Disease in Patients With Idiopathic Inflammatory Myopathy: A Series of 474 Patients.pdf

Objective: This study was conducted to identify the characteristics and prognosis of rapidly progressive interstitial lung disease (RP-ILD) in idiopathic inflammatory myopathy (IIM) and to assess the predictors for poor survival of RP-ILD in IIM.Methods: A total of 474 patients with IIM were enrolled retrospectively according to medical records from Peking University People's Hospital. Clinical and laboratory characteristics recorded at the diagnosis of patients with RP-ILD and chronic ILD (C-ILD) were compared. The Kaplan–Meier estimator and univariate and multivariate analyses were used for data analysis.Results: ILD was identified in 65% (308/474) of patients with IIM. Patients with ILD were classified into two groups based on lung features: RP-ILD (38%, 117/308) and C-ILD (62%, 191/308). RP-ILD resulted in significantly higher mortality in IIM compared with C-ILD (27.4 vs. 7.9%, P < 0.05). In this study, by comparing IIM patients with and without RP-ILD, a list of initial predictors for RP-ILD development were identified, which included older age at onset, decreased peripheral lymphocytes, skin involvement (periungual erythema, skin ulceration, and subcutaneous/mediastinal emphysema), presence of anti-MDA5 antibody, serum tumor markers, etc. Further multivariate Cox proportional hazards model analysis identified that anti-MDA5 positivity was an independent risk factor for mortality due to RP-ILD (P < 0.05), and lymphocytes

研究目的：本项研究旨在探究特发性炎症性肌病（IIM）中快速进展性间质性肺疾病（RP-ILD）的特征及其预后，并评估IIM中RP-ILD不良生存的预测因素。研究方法：共纳入北京大学人民医院医疗记录中的474例IIM患者进行回顾性研究。比较了RP-ILD和慢性间质性肺疾病（C-ILD）患者在诊断时的临床和实验室特征。采用Kaplan-Meier估计器和单因素及多因素分析进行数据分析。研究结果：在IIM患者中，间质性肺疾病的发生率为65%（308/474）。根据肺部特征，将ILD患者分为两组：RP-ILD组（38%，117/308）和C-ILD组（62%，191/308）。与C-ILD相比，RP-ILD在IIM患者中导致显著更高的死亡率（27.4% vs. 7.9%，P < 0.05）。在本研究中，通过比较具有和未具有RP-ILD的IIM患者，确定了RP-ILD发展的初始预测因素列表，包括发病年龄较大、外周淋巴细胞减少、皮肤受累（甲周红斑、皮肤溃疡和皮下/纵隔气肿）、抗MDA5抗体阳性、血清肿瘤标志物等。进一步的多变量Cox比例风险模型分析显示，抗MDA5阳性是RP-ILD死亡率的独立风险因素（P < 0.05），且淋巴细胞计数等。