This open-label extension trial (InPedILD-ON) assessed the longer-term safety and tolerability of nintedanib in 48 children and adolescents (ages 6–17, mean age 13.7 years) with fibrosing ILDs, with a median treatment exposure of 61.5 weeks. Exploratory lung function outcomes (FVC % predicted, SpO2) were also collected up to Week 52.
Diarrhea was the most frequent adverse event (31.3% of patients; rate 50.4 per 100 patient-years), but no patient stopped nintedanib because of it. Seven of 48 patients (14.6%) discontinued for various reasons; only one stopped due to an adverse event (weight decrease). Mean FVC % predicted change at Week 52 was −1.0% (SE 1.4; n=26), and mean SpO2 change was +0.3% (SE 0.8; n=30). No premature epiphyseal closure or dental root stunting/acceleration was found on imaging.
- Small sample (n=48) with no comparator arm, limiting efficacy conclusions. - Selection bias likely: patients tolerating nintedanib in InPedILD were more likely to roll over into InPedILD-ON. - FVC data were available for only 26 of 48 patients at Week 52, and measurements showed large variability.
Nintedanib appears tolerable in pediatric fibrosing ILD over roughly 14 months, with a safety profile consistent with the parent InPedILD trial; diarrhea is common but manageable. Clinicians should monitor weight closely in children on nintedanib, as insufficient weight gain or loss can necessitate discontinuation.
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