LUNG function declined more slowly after nintedanib initiation in a national study of patients with progressive pulmonary fibrosis. The estimated annual loss in forced vital capacity (FVC) fell by more than half after treatment began. Even with the slower decline, estimated survival at two years was 70.7%.
The retrospective study included 359 patients who started nintedanib at Denmark’s four tertiary interstitial lung disease centers between 2022 and 2024. The median age was 72 years. Interstitial lung disease associated with systemic autoimmune rheumatic disease was the most common underlying diagnosis (38%), followed by fibrotic hypersensitivity pneumonitis (28%). At treatment initiation, median FVC was 69% of predicted, and median diffusing capacity for carbon monoxide (DLCO) was 42% of predicted.
Annual FVC decline decreased from 224 mL before nintedanib initiation to 92 mL afterward, a difference of 132 mL per year (95% CI, 80–184 mL per year; P<.001). The annual rate of decline in DLCO did not change significantly. Gastrointestinal adverse effects were the most common reason for stopping nintedanib.
During a median follow-up of 25.2 months, 115 patients died. Estimated survival was 94.1% at six months, 84.8% at one year, and 70.7% at two years. Older age, lower baseline FVC, and lower baseline DLCO were each independently associated with higher mortality. A usual interstitial pneumonia pattern on CT had no statistically significant association with mortality in the adjusted analysis.
The before-and-after comparison had no untreated control group, which limits what the study can establish about nintedanib’s effects on lung function or survival. Follow-up continued after patients stopped treatment, so the estimates describe lung function after starting nintedanib across the cohort. Patients with the fastest progression may also have been less likely to contribute follow-up lung function measurements, potentially understating the rate of decline.
For clinicians discussing prognosis with patients who have progressive pulmonary fibrosis, baseline FVC and DLCO help put the slower decline in context. Both measures carried prognostic information, and nearly three in ten patients were estimated to have died within two years of starting treatment.
Reference
Makholm N et al. Real-world outcomes in PPF treated with nintedanib: a nationwide cohort study. ERJ Open Res. 2026. doi:10.1183/23120541.00631-2026.
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