Nintedanib for Interstitial Lung Disease

No longer recruiting at 51 trial locations

Overseen By Boehringer Ingelheim

Age: < 18

Sex: Any

Trial Phase: Phase 3

Sponsor: Boehringer Ingelheim

Must be taking: Nintedanib

Must not be taking: Anticoagulants, Antiplatelets

Disqualifiers: Liver disease, Hypertension, Cardiovascular, others

No Placebo Group All trial participants will receive the active study treatment (no placebo)

Pivotal Trial (Near Approval) This treatment is in the last trial phase before FDA approval

Prior Safety Data This treatment has passed at least one previous human trial

Breakthrough Therapy This drug has been fast-tracked for approval by the FDA given its high promise

Compensation: Varies

Number of Visits: 15

Duration: At least 3 years for rollover participants, at least 1.5 years for new participants

What You Need to Know Before You Apply

What is the purpose of this trial?

This trial tests a medicine called nintedanib to evaluate its effectiveness for children and teens with interstitial lung disease (ILD) that leads to lung fibrosis. The goal is to assess long-term tolerance in children. It targets those aged 6 to 17 with fibrosing ILD who either participated in a previous study called InPedILD or are new to this trial. Participants will take nintedanib capsules twice daily and visit the study site regularly to monitor health. As a Phase 3 trial, this study is the final step before FDA approval, offering participants the opportunity to help bring a new treatment to market.

Do I need to stop my current medications to join the trial?

The trial protocol does not specify if you need to stop taking your current medications. However, you cannot take any drugs that might interfere with the trial, according to the investigator's assessment.

Is there any evidence suggesting that nintedanib is likely to be safe for children and adolescents?

Research has shown that nintedanib is generally well-tolerated by people with lung diseases. Studies have identified stomach issues, such as nausea and diarrhea, as the most common side effects. Patients frequently reported these complaints. One study found that serious problems occurred in about 44% of people taking nintedanib, compared to about 50% of those not taking it. This indicates that while serious side effects can occur, they are not more common than in people using other treatments.

Nintedanib is already approved for adults with various types of lung fibrosis, suggesting it is considered safe for adult use. However, this trial aims to assess how well children and teens tolerate it over a longer period. The existing data provides a good starting point for understanding its safety in younger people. ^{1 2 3 4 5}

Why do researchers think this study treatment might be promising?

Unlike the standard treatments for interstitial lung disease, which typically include corticosteroids and immunosuppressants, nintedanib works by inhibiting multiple tyrosine kinases. This mechanism helps to halt the scarring process in the lungs. Researchers are excited about nintedanib because it targets the fibrotic process directly, potentially slowing disease progression more effectively than current options. Additionally, its ability to address a broad range of growth factors involved in fibrosis makes it a promising candidate for improving lung function in these patients.

What is the effectiveness track record for nintedanib in treating lung fibrosis?

Research shows that nintedanib can slow the progression of lung scarring in various types of interstitial lung disease (ILD). Studies have found that it significantly slows the loss of lung function, measured by forced vital capacity (FVC), in patients with idiopathic pulmonary fibrosis (IPF) and other progressive ILDs. In patients with ILD linked to systemic sclerosis, nintedanib also reduced the rate of lung function decline over a year. Additionally, real-world evidence supports its effectiveness and safety in treating other types of fibrosing ILDs. While its use in children is still under investigation, these findings suggest that nintedanib may also help slow lung scarring in young patients. Participants in this trial will receive nintedanib, either as new enrollees or as rollovers from the InPedILD® study. ^{2 3 5 6 7}

Are You a Good Fit for This Trial?

This trial is for children and adolescents aged 6-17 with fibrosing interstitial lung disease. They must have participated in a prior study or meet specific health criteria, including normal organ function tests and not being on conflicting medications. Girls able to have children must use effective birth control.

Inclusion Criteria

For roll-over patients from the InPedILD® study: Patients who completed the InPedILD® trial as planned and who did not permanently prematurely discontinue study treatment

New patients must have a lung function measurement called Forced Vital Capacity (FVC) of at least 25% at their second visit.

I have been diagnosed with fibrosing ILD in the last year.

Exclusion Criteria

I am not pregnant, nursing, or planning to become pregnant during the trial.

I need or want to take medication that may not be safe with the trial.

I am willing and able to follow the trial procedures and take the study medication.

Timeline for a Trial Participant

Screening

Participants are screened for eligibility to participate in the trial

2-4 weeks

Treatment

Participants take nintedanib capsules twice a day for long-term safety and tolerability assessment

At least 3 years for rollover participants, at least 1.5 years for new participants

Approximately 15 visits over 3 years, then every 3 months

Follow-up

Participants are monitored for safety and effectiveness after treatment

6 months

What Are the Treatments Tested in This Trial?

Interventions

• Nintedanib

Trial Overview The trial evaluates the long-term safety of Nintedanib (Ofev®) in treating lung fibrosis in young patients. Participants will take Nintedanib capsules twice daily for at least six months, with regular visits to monitor their health.

How Is the Trial Designed?

Group I: Patients rolling over from the InPedILD® study Experimental Treatment 1 Intervention

Group II: Patients newly enrolled in this study Experimental Treatment 1 Intervention

Who Is Running the Clinical Trial?

Boehringer Ingelheim

Lead Sponsor

Trials

2,566

Recruited

16,150,000+

Published Research Related to This Trial

In a study of 5578 Japanese patients with idiopathic pulmonary fibrosis (IPF), approximately 50% discontinued nintedanib treatment within 12 months, primarily due to adverse drug reactions (ADRs) such as hepatic function abnormalities and diarrhea.

Patients who continued nintedanib showed a smaller decline in lung function (forced vital capacity) compared to those who discontinued, indicating that ongoing treatment may help preserve lung function despite the risk of adverse effects.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Real-World Safety and Tolerability of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis: Interim Report of a Post-Marketing Surveillance in Japan. Ogura, T., Inoue, Y., Azuma, A., et al.[2023]

In the INBUILD trial involving 170 patients with fibrosing autoimmune disease-related interstitial lung diseases, nintedanib significantly slowed the decline in lung function (measured by forced vital capacity) compared to placebo, with a difference of 102.7 ml/year over 52 weeks.

While nintedanib was effective, it was associated with a higher incidence of adverse events, particularly diarrhea, affecting 63.4% of patients, though most side effects were manageable.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Nintedanib in Patients With Autoimmune Disease-Related Progressive Fibrosing Interstitial Lung Diseases: Subgroup Analysis of the INBUILD Trial. Matteson, EL., Kelly, C., Distler, JHW., et al.[2022]

In a study involving 109 patients (27 with systemic sclerosis-related interstitial lung disease and 82 with idiopathic pulmonary fibrosis), the safety profile of nintedanib was found to be similar for both groups, with no significant differences in the frequency of adverse effects.

Patients with systemic sclerosis-related interstitial lung disease required dosage reductions or interruptions of nintedanib sooner than those with idiopathic pulmonary fibrosis, highlighting the need for effective management strategies, such as dietary adjustments and probiotics, to maintain treatment efficacy.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Real life data on nintedanib safety: idiopathic pulmonary fibrosis versus systemic sclerosis-interstitial lung disease and strategies adopted to manage adverse effects. Di Battista, M., Tavanti, L., Pistelli, F., et al.[2023]

Citations

1. pmc.ncbi.nlm.nih.gov pmc.ncbi.nlm.nih.gov/articles/PMC9405731/

The Effectiveness of Nintedanib in Patients with Idiopathic ... Results: nintedanib treatment significantly reduced the FVC decline rate in IPF and PF-ILD subgroups, but not in FPF subjects. No significant ...

2. publications.ersnet.org publications.ersnet.org/content/erj/59/3/2004538

Nintedanib in progressive interstitial lung diseases: data from ... Nintedanib reduces the rate of progression of interstitial lung disease (ILD) over 52 weeks in patients with idiopathic pulmonary fibrosis (IPF).

3. nejm.org nejm.org/doi/full/10.1056/NEJMoa1903076

Nintedanib for Systemic Sclerosis–Associated Interstitial ... The results showed that the rate of decline in FVC over 52 weeks among patients with ILD associated with systemic sclerosis was lower for those who received ...

4. sciencedirect.com sciencedirect.com/science/article/abs/pii/S0049017225000812

Real-world evidence of the antifibrotic nintedanib in ... Nintedanib seems to be effective and relatively safe in progressive fibrosing RA-ILD despite clinical differences with the INBUILD trial.

5. clinicaltrials.gov clinicaltrials.gov/study/NCT02999178

NCT02999178 | Efficacy and Safety of Nintedanib in ... The aim of the current study is to investigate the efficacy and safety of nintedanib over 52 weeks in patients with Progressive Fibrosing Interstitial Lung ...

6. pubmed.ncbi.nlm.nih.gov pubmed.ncbi.nlm.nih.gov/39466587/

Safety and Tolerability of Nintedanib in Patients with ... The results of clinical trials showed that the most frequent adverse events seen in patients treated with nintedanib are gastrointestinal events, particularly ...

7. respiratory-research.biomedcentral.com respiratory-research.biomedcentral.com/articles/10.1186/s12931-022-01974-2

Safety and tolerability of nintedanib in ... - Respiratory Research Serious adverse events were reported in 44.3% of patients in the nintedanib group and 49.5% of patients in the placebo group. The most common ...