Nintedanib is widely used to slow disease progression and prevent acute exacerbations in patients with idiopathic pulmonary fibrosis (IPF) and progressive fibrosing interstitial lung disease (PF-ILD). We retrospectively analysed patients who initiated nintedanib for IPF or PF-ILD between August 2019 and July 2023 across 15 institutions in Japan, focusing on prognosis and disease progression. Patients were divided into two groups based on whether they survived for ≥ 3 years after nintedanib initiation, and their characteristics were compared. We also evaluated factors associated with annual forced vital capacity (FVC) decline and acute exacerbations. A total of 413 patients (171 with IPF and 242 with PF-ILD) were included. Median survival was 1,177 days for IPF and 1,268 days for PF-ILD, with no significant difference (P = 0.20). Patients surviving < 3 years were older (75.0 vs. 71.0 years), more frequently had resting arterial oxygen saturation (SpO2) < 95% (44.5% vs. 18.0%), and lower body mass index (BMI) (21.9 vs. 24.1 kg/m2). Additionally, patients with a BMI < 22 kg/m2 showed greater annual relative FVC decline. In conclusion, patients with IPF and PF-ILD showed comparable outcomes following nintedanib treatment. Age ≥ 65 years, resting SpO2 < 95%, and BMI < 22 kg/m2 were associated with shorter survival after nintedanib initiation.
Supplementary Information: The online version contains supplementary material available at 10.1038/s41598-025-34071-7.
Keywords: Fibrosis; Interstitial lung disease; Nintedanib; Prognostic factor; Survival; Treatment.