New Data Support Potential Use of Nintedanib in Children, Adolescents with Fibrosing Interstitial Lung Disease


Nintedanib is a tyrosine kinase inhibitor targeting key receptors involved in signaling pathways that led to pulmonary fibrosis.

A weight-based dosing regimen of nintedanib (Ofev, Vargatef; Boehringer Ingelheim) in children and adolescents with fibrosing interstitial lung disease (ILD) was found to have an acceptable safety and tolerability profile with no new safety signals observed when compared to adult patients with idiopathic pulmonary fibrosis (IPF), other progressive fibrosing ILD (PF-ILD), and systemic sclerosis-associated interstitial lung disease (SSc-ILD) in the phase 3 InPedILD trial.

The InPedILD trial assessed the dosing and safety profile of nintedanib in children and adolescents between 6 and 17 years of age with clinically significant fibrosing ILD over 24 weeks, which showed encouraging data for both primary endpoints. The results were published in the European Respiratory Journal.

“Based on the nintedanib mode of action, preclinical evidence and the clinical benefit in adults, there was a compelling rationale for examining its effect in children living with interstitial lung disease,” said coordinating investigator, professor Robin Deterding, MD, director of the Breathing Institute, Children's Hospital Colorado, in a press release. “This trial supports its potential use as a treatment with an acceptable safety profile for children and adolescents, for whom no approved evidence-based therapies exist.”

Nintedanib is a tyrosine kinase inhibitor targeting key receptors involved in signaling pathways that led to pulmonary fibrosis. It has been approved in more than 80 countries for the treatment of patients living with IPF, SSc-ILD, and other chronic fibrosing ILDs with a progressive phenotype.

In September 2019, nintedanib became the first treatment approved by the FDA for patients with ILD associated with SSc-ILD. Nintendanib was initially approved in October 2014 for idiopathic pulmonary fibrosis, which is another interstitial lung condition. Approximately 25% of patients develop significant pulmonary involvement within 3 years of an ILD diagnosis.

The InPedILD trial results showed that the weight-based dosing regimen of nintedanib in children and adolescents with fibrosing ILD resulted in comparable exposure to adult patients with fibrosing ILD. The most common adverse event (AE) seen in adults associated with nintedanib was diarrhea. All reported diarrhea AEs could be resolved without premature discontinuation of trial medication. The results have led to the process of regulatory applications being submitted to the European Medicines Agency and FDA.

“While childhood interstitial lung diseases are very rare, their impact on children, teenagers and their loved ones can be devastating,” said Susanne Stowasser, MD, associate head of Medicine Pulmonology at Boehringer Ingelheim, in a press release. “The findings from InPedILD help meet the urgent need for well-characterized therapies for these children and adolescents living with ILD. These data further support Boehringer Ingelheim’s ongoing commitment to address unmet needs and advance research for people across all generations living with pulmonary fibrosis.”


New data from Boehringer Ingelheim support the potential use of nintedanib in children and adolescents with fibrosing interstitial lung disease. Boehringer Ingelheim. September 5, 2022. Accessed September 7, 2022.

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