Real-World Experience with Nintedanib in a Progressive Pulmonary Fibrosis Cohort

Area of Science:

• Pulmonology
• Pharmacology
• Clinical Medicine

Background:

• Nintedanib is approved for progressive pulmonary fibrosis (PPF) based on clinical trials.
• Real-world data is crucial for understanding nintedanib's effectiveness and safety in diverse PPF patient populations.

Purpose of the Study:

• To evaluate nintedanib's effect on forced vital capacity (FVC) and diffusing lung capacity for carbon monoxide (DLCO) after one year.
• To compare the annual rate of decline in FVC, DLCO, and 6-minute walk distance (6MWD) before and after nintedanib initiation.
• To assess safety, tolerability, adverse events, and impact on immunosuppressant use, dyspnoea, and survival.

Main Methods:

• Retrospective, observational cohort study at a single center.
• Included consecutive patients with non-IPF fibrotic interstitial lung disease (ILD) treated with nintedanib.
• Analyzed changes in FVC, DLCO, 6MWD slopes, safety, and secondary outcomes over one year.

Main Results:

• Most patients (63.4% FVC, 82.5% DLCO) stabilized or improved lung function after one year.
• A non-statistically significant reduction in the annual decline rate (slope) for FVC and DLCO was observed post-treatment.
• Dyspnoea improved in 23.2% of patients, and immunosuppressant use decreased.
• Common side effects included diarrhoea (60%), hepatotoxicity (23.6%), and asthenia (12.7%); 15 patients discontinued treatment.

Conclusions:

• Nintedanib demonstrated stabilization or improvement in FVC and DLCO in a real-world PPF cohort.
• A trend towards reduced FVC decline and improved symptoms was noted, supporting nintedanib's role in managing PPF.
• Nintedanib's safety profile was consistent with clinical trials, requiring monitoring for adverse events like diarrhoea and hepatotoxicity.