Real-world use of nintedanib for the treatment of interstitial lung disease with progressive pulmonary fibrosis

Abstract

Background

Patients with non-idiopathic interstitial lung diseases can develop progressive pulmonary fibrosis (PPF-ILD). The study objectives were to define the profile of patients with PPF-ILD treated with nintedanib and to assess the effectiveness and safety of this drug in a real-world setting.

Methods

This was a multicenter, prospective, observational study of adult patients with PPF-ILD that initiated treatment with nintedanib in ten Andalusian hospitals (Spain). Demographic data, smoking habit, underlying disease, and diagnosis criteria were recorded. Pulmonary function test results, the dyspnea scale score, and the number of hospitalizations related to PPF-ILD were evaluated at baseline and after 6 and 12 months. Adverse events were recorded.

Results

Between July 2021 and March 2023, a total of 145 patients entered the study and were followed up until March 2024; 55% were men and the mean age was 66.6 ± 11.5 years. PPF-ILD was diagnosed based on clinical, radiological and pulmonary function test findings in 97 patients (66.9%). The mean ± SD duration of nintedanib therapy was 13.3 ± 10.1 months. Dyspnea improved, although the difference was not statistically significant. FVC % and D_LCO % stabilized. The number of hospitalizations related to PPF-ILD was reduced (p < 0.0001) after 12 months of therapy. Diarrhea was the most common adverse event.

Conclusions

In this real-world study, the profile of PPF-ILD patients treated with nintedanib was consistent with the approved therapeutic indications. Nintedanib reduced the decline in pulmonary function and the number of hospitalizations, and it was well tolerated. At 12 months, 75.1% of patients remained on treatment; treatment discontinuation occurred in 24.9% due to adverse events, death, or lung transplantation.