Efficacy and Tolerability of Nintedanib in Idiopathic-Inflammatory-Myopathy-Related Interstitial Lung Disease: A Pilot Study

作者：Junyu Liang, Heng Cao, Yang Yang, Yini Ke, Ye Yu, Chuanyin Sun, Lihuan Yue, Jin Lin · 发表于：Frontiers in Medicine · 年份：2021 · DOI：10.3389/fmed.2021.626953 · 被引用次数：40 · 研究领域：Inflammatory Myopathies and Dermatomyositis、Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis、Eosinophilic Disorders and Syndromes

Objectives: To initially clarify the efficacy and tolerability of nintedanib in patients with idiopathic-inflammatory-myopathy-related interstitial lung disease (IIM-ILD). Methods: A retrospective, real-world analysis was conducted in IIM-ILD patients who regularly received outpatient visit or hospitalization from January 2018 to March 2020 in three centers. And the patients were divided into two groups depending on presence or absence of nintedanib therapy. Comparisons, Kaplan-Meier survival analysis and propensity score matching were made to identify difference in time to death from any cause, incidence of rapidly progressive interstitial lung disease (RP-ILD) and comorbidity of pulmonary infection between the two groups. The following logistic regression analyses and Cox proportional-hazard regression analyses were used to verify the therapeutic value of nintedanib as well as clinical significance of other factors. Adverse events were descriptively recorded. Results: Thirty-six patients receiving nintedanib therapy and 115 patients without use of nintedanib were included. Before and after propensity score matching, the primary comparisons revealed better survival ( P = 0.015, P = 0016, respectively) and lower incidence of RP-ILD ( P = 0.017, P = 0.014, respectively) in patients with nintedanib therapy. Logistic regression analysis identified that disease activity ( P < 0.001), percent-predicted diffusing capacity of the lung for carbon monoxide (DLCO%, P = 0.036), n...