BACKGROUND: In the InPedILD trial, nintedanib had an acceptable safety profile in children and adolescents (aged 6-17 years) with fibrosing ILDs. The open-label extension of the InPedILD trial, InPedILD-ON, is assessing the longer-term safety of nintedanib in these patients. METHODS: Patients who completed the InPedILD trial on treatment and had a transition period of 12 weeks, and new patients aged 6-17 years with fibrosing ILDs, entered InPedILD-ON as "new patients." All patients received open-label nintedanib in InPedILD-ON. RESULTS: Forty-eight patients received nintedanib in InPedILD-ON. At baseline, mean (SD) age was 13.7 (3.2) years, and FVC was 59.4 (21.6) % predicted. At this data snapshot, median exposure to nintedanib in InPedILD-ON was 61.5 weeks. Diarrhea was the most frequent adverse event, reported at a rate of 50.4 per 100 patient-years. Seven patients discontinued nintedanib, one for each of the following reasons: adverse event (weight decrease), change of residence, burden of study procedures, pregnancy planning, clinical deterioration, other treatment option available, no reason given. Mean (SE) change in FVC % predicted at Week 52 (n = 26) was -1.0 (1.4). Mean (SE) change in SpO(2) (%) on room air at rest at Week 52 (n = 30) was 0.3 (0.8). CONCLUSION: The adverse event profile of nintedanib in InPedILD-ON was generally consistent with that reported in the InPedILD trial, supporting the tolerability of nintedanib in children and adolescents with fibrosing ILDs.
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