Idiopathic 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.The impact of nintedanib therapy on lung
function, survival, and adverse events was analyzed longitudinally.Twenty-two out
of 103 patients were included in the analysis (group 1: N = 10; male/female = 6:4,
age 62.6 ± 10.8 years and group 2: N = 12; male/female = 3:9, age 65.7 ± 11.6 years).
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 444 days for group 1 and 476 days 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.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 1 year in both groups.