Early administration of nintedanib after the onset of acute exacerbation in patients with interstitial lung disease

Abstract

<b>Background:</b> Nintedanib (NTD) is an anti-fibrotic agent for idiopathic pulmonary fibrosis (IPF) or progressive fibrosing interstitial lung disease (PF-ILD); it inhibits triple receptors, including vascular endothelial growth factor (VEGF) receptor. VEGF was reportedly associated with the pathogenesis of acute exacerbation of IPF and PF-ILD (AE). However, there have been few studies regarding the effect of NTD on AE period. <b>Aim:</b> We aimed to investigate the efficacy and safety of NTD for AE. <b>Methods:</b> We retrospectively collected data of 95 patients who developed AE between April 2012 and January 2022. Among the patients, 14 who had received NTD within three months before AE and 19 who died within seven days after developing AE were excluded from this study. Eleven of 62 patients who started treatment with NTD within a month after developing AE were placed in the NTD (N) group. The other patients were placed in the no NTD (No-N) group. The efficacy and safety of NTD were compared between the two groups. <b>Results:</b> There were no significant differences in the P/F ratio at AE development, and other treatments for AE between the two groups. At 90 days from AE development, the survival rate was significantly higher in the N group than in the Non-N group (81.8% vs. 43.1%, p=0.020). The median survival time was significantly longer in the N group than in the Non-N group (213 days vs. 65 days, p=0.030, generalized Wilcoxon test).&nbsp;Although one patient developed a severe adverse effect associated with the NTD treatment in the N group, no patients had to discontinue NTD treatment. <b>Conclusion:</b> Additional NTD treatment may be associated with better clinical outcomes without severe adverse effects.