Related Experiment Videos

Continued Nintedanib Treatment in Children and Adolescents With Fibrosing ILDs: Data From InPedILD-ON

Robin Deterding1,2, Lisa R Young3, Emily M DeBoer1,2

  • 1Section of Pediatric Pulmonary and Sleep Medicine, Department of Pediatrics, University of Colorado Denver, Denver, Colorado, USA.

Pediatric Pulmonology
|June 22, 2026
PubMed

Insights

Nintedanib demonstrated acceptable long-term safety and tolerability in pediatric patients with fibrosing interstitial lung diseases (ILDs). The adverse event profile was consistent with previous findings in this young population.

Area of Science:

  • Pediatric Pulmonology
  • Pharmacology
  • Clinical Trials

Background:

  • The InPedILD trial established nintedanib's safety in children and adolescents (6-17 years) with fibrosing interstitial lung diseases (ILDs).
  • The InPedILD-ON trial extends this by evaluating the longer-term safety and tolerability of nintedanib in this patient group.

Purpose of the Study:

  • To assess the long-term safety and tolerability of nintedanib in pediatric patients with fibrosing ILDs.
  • To evaluate adverse events and clinical outcomes during extended nintedanib treatment in children and adolescents.

Main Methods:

  • Patients from the InPedILD trial (rollover) and new pediatric patients (6-17 years) with fibrosing ILDs were enrolled.
  • All participants received open-label nintedanib, with safety and efficacy data collected over an extended period.

Main Results:

  • Forty-eight patients received nintedanib, with a median exposure of 61.5 weeks.
  • Diarrhea was the most frequent adverse event (50.4 per 100 patient-years).
  • Mean change in FVC % predicted and SpO2 at Week 52 were -1.0% and +0.3%, respectively.

Conclusions:

  • Nintedanib exhibited an adverse event profile consistent with the initial InPedILD trial.
  • These findings support the continued tolerability of nintedanib for long-term use in pediatric patients with fibrosing ILDs.
Abstract

Keywords:
disease progressiondrug side effectspediatricspulmonary fibrosisrespiratory function tests

Related Concept Videos

Drugs for Treatment of Crohn's Disease in IBD Using Immunomodulatory Agents01:29

Drugs for Treatment of Crohn's Disease in IBD Using Immunomodulatory Agents

Crohn's disease is an inflammatory bowel disorder marked by chronic inflammation of the GI tract. Various treatment strategies for Crohn's disease are employed, such as immunomodulatory agents, glucocorticoids, and biologics or anti-TNF therapy. Azathioprine (Imuran), a commonly used immunomodulatory drug for Crohn's disease, is converted in the body to mercaptopurine, which inhibits purine biosynthesis and cell proliferation. Both are utilized in severe cases of Inflammatory Bowel Disease...
Inflammatory Bowel Disease IV: Pharmacological Management01:29

Inflammatory Bowel Disease IV: Pharmacological Management

Upon diagnosis, managing Inflammatory Bowel Disease (IBD) involves addressing several crucial aspects. The primary goals include resting the bowel, correcting malnutrition, and providing symptomatic relief. Resting the bowel may consist of medications to reduce inflammation and promote healing. Correcting malnutrition is essential, often requiring dietary adjustments and nutritional supplements. Symptomatic relief aims to ease pain, diarrhea, and other discomforts in IBD.
Pharmacologic...
Drugs for Treatment of Crohn's Disease in IBD Using Biologic Agents: Anti-TNF01:24

Drugs for Treatment of Crohn's Disease in IBD Using Biologic Agents: Anti-TNF

Tumor Necrosis Factor (TNF), a proinflammatory cytokine, contributes significantly to the inflammation seen in Crohn's disease. It exists as soluble TNF and membrane-bound TNF, with actions mediated through TNF receptors (TNFR). TNFR activation leads to the release of proinflammatory cytokines, T-cell activation, collagen production, and leukocyte migration, all contributing to inflammation in Crohn's disease. Anti-TNF monoclonal antibodies, namely infliximab (Remicade), adalimumab (Humira),...
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists01:18

Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists

Endothelins (ETs) are potent vasoactive peptides critical in the human body's various physiological and pathological processes. One of the most promising therapeutic strategies for treating pulmonary arterial hypertension (PAH) involves counteracting the effects of these endothelins using a class of drugs known as endothelin receptor antagonists.
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme (ECE). Of...
Drugs for Treatment of Crohn's Disease in IBD Using Glucocorticoids01:21

Drugs for Treatment of Crohn's Disease in IBD Using Glucocorticoids

Glucocorticoids, a class of anti-inflammatory drugs, are pivotal in treating moderate to severe Crohn's disease by inducing remission. They exhibit their anti-inflammatory action by inhibiting the production of inflammatory cytokines such as tumor necrosis factor (TNF)-α, interleukin (IL)-1, and chemokines like IL-8. In addition, they reduce the expression of inflammatory cell adhesion molecules and inhibit gene transcription of nitric oxide synthase, phospholipase A2, cyclooxygenase-2 (COX-2),...
Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors01:28

Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors

Phosphodiesterase 5 (PDE5) inhibitors are potent enzymes that function to hydrolyze cyclic nucleotides to their corresponding 5' monophosphates. Their unique biochemical properties have been applied in treating Pulmonary Arterial Hypertension (PAH).
Among the PDE5 inhibitors, sildenafil (Revatio) stands out as a competitive and selective inhibitor. It operates by elevating cellular levels of cGMP and augmenting signaling through the cGMP-PKG pathway, promoting vasodilation. Upon oral...