Long-Term Effects and Adverse Events of Nintedanib Therapy in Idiopathic Pulmonary Fibrosis Patients with Functionally Advanced Disease

IntroductionIdiopathic pulmonary fibrosis (IPF) is one of the most common interstitial lung diseases with limited survival. The effect of IPF therapy in patients with severely impaired lung function is not well established. The aim of this study was to characterize IPF patients with a forced vital capacity (FVC)<50% (group 1) and FVC 50-60% predicted (group 2) and analyze the effect and adverse events of nintedanib in Hungarian patients diagnosed between April 2015 and July 2017.MethodsThe impact of nintedanib therapy on lung function, survival, and adverse events was analyzed longitudinally.ResultsTwenty-two out of 103 patients were included in the analysis (group 1: N=10; male/female=6:4, age 62.610.8years and group 2: N=12; male/female=3:9, age 65.7 +/- 11.6years). Eighteen patients were treated with nintedanib (8 in group 1, 10 in group 2); treatment stabilized lung function in 42% and 50%, respectively, in the two groups. Median survival was 444days for group 1 and 476days for group 2. Adverse events were less common than in clinical trials; dose reduction was necessary in three cases, drug discontinuation in two cases. No differences between groups were identified regarding clinical parameters and radiological pattern; however, hypertension as comorbidity was more common in group 1 patients.Conclusions Nintedanib therapy was effective and well tolerated even among patients with severely impaired lung function. Longitudinal follow-up confirmed high mortality in patients with very severe and severe IPF; however, median survival was meaningful as it exceeded 1year in both groups.