Nintedanib Halves Annual FVC Decline in Progressive Pulmonary Fibrosis

LUNG function declined more slowly after nintedanib initiation in progressive pulmonary fibrosis patients, according to a nationwide study published in the ERJ Open Res.

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 across the cohort was 72 years.

Interstitial lung disease associated with systemic autoimmune rheumatic disease accounted for 38% of underlying diagnoses. Fibrotic hypersensitivity pneumonitis made up 28% of the initial cases.

At the start of treatment, the median forced vital capacity was 69% of predicted values. The median diffusing capacity for carbon monoxide sat at 42% of predicted.

The estimated annual loss in forced vital capacity fell by more than half after treatment began. Annual decline dropped from 224 mL before nintedanib initiation to 92 mL afterward.

That shift marked a difference of 132 mL per year, with a 95% confidence interval ranging from 80 to 184 mL per year. Researchers recorded a P value of less than .001 for the change.

The annual rate of decline in diffusing capacity for carbon monoxide showed no significant change. Gastrointestinal adverse effects emerged as the most common reason patients stopped taking nintedanib.

During a median follow-up period of 25.2 months, 115 patients died. Estimated survival reached 94.1% at six months and 84.8% at one year.

Estimated survival dropped to 70.7% at two years. Older age, lower baseline forced vital capacity, and lower baseline diffusing capacity for carbon monoxide each linked independently to higher mortality.

A usual interstitial pneumonia pattern on computed tomography scans had no statistically significant association with mortality in the adjusted analysis. The before-and-after comparison lacked an untreated control group.

That design limitation restricts what the study can establish about nintedanib’s direct effects on lung function or survival. Follow-up continued even after patients stopped treatment, meaning the estimates describe lung function across the cohort after starting nintedanib.

Patients with the fastest disease progression may have been less likely to contribute follow-up lung function measurements. That dynamic could potentially understate the actual rate of decline.

Clinicians discussing prognosis with progressive pulmonary fibrosis patients use baseline forced vital capacity and diffusing capacity for carbon monoxide to contextualize the slower decline. Both measures carried prognostic information in the data.

Nearly three in ten patients were estimated to have died within two years of starting treatment. Makholm and colleagues conducted the research as a nationwide cohort study on real-world outcomes in progressive pulmonary fibrosis treated with nintedanib.

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