Nintedanib for Interstitial Lung Disease

Recruiting at 38 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

56 Participants Needed

Trial Summary

What is the purpose of this trial?

This trial tests the medicine nintedanib in children and teens with lung disease that causes scarring. The goal is to see if long-term use of nintedanib is safe and effective for them. Participants take the medicine and visit the study site for health check-ups. Nintedanib has been shown to slow disease progression and is well tolerated in patients.

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.

What data supports the effectiveness of the drug Nintedanib for Interstitial Lung Disease?

Research shows that Nintedanib can slow the progression of lung diseases like idiopathic pulmonary fibrosis (IPF) by reducing the decline in lung function. It has also been effective in treating interstitial lung disease related to autoimmune diseases and systemic sclerosis, suggesting it may help with other similar lung conditions. ^{1 2 3 4 5}

Is Nintedanib safe for humans?

Nintedanib is generally safe for humans, but it can often cause stomach-related side effects. Studies show that some people may need to adjust their dose or stop taking it due to these side effects. ^{6 7 8 9 10}

How does the drug Nintedanib differ from other treatments for interstitial lung disease?

Nintedanib is unique because it is a triple-tyrosine kinase inhibitor that targets specific growth factors involved in lung fibrosis, which helps slow the progression of lung damage and improve quality of life. Unlike traditional treatments that may focus on suppressing the immune system, Nintedanib directly interferes with the pathways that lead to fibrosis, making it a novel option for conditions like idiopathic pulmonary fibrosis and systemic sclerosis-associated interstitial lung disease. ^{2 4 11 12 13}

Eligibility Criteria

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

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

Treatment Details

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.

Participant Groups

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+

Findings from Research

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]

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]

References

1. Australia pubmed.ncbi.nlm.nih.gov

Outcomes of patients with advanced idiopathic pulmonary fibrosis treated with nintedanib or pirfenidone in a real-world multicentre cohort. [2021]

2. United States pubmed.ncbi.nlm.nih.gov

Nintedanib in Patients With Autoimmune Disease-Related Progressive Fibrosing Interstitial Lung Diseases: Subgroup Analysis of the INBUILD Trial. [2022]

3. Switzerland pubmed.ncbi.nlm.nih.gov

Insights from the German Compassionate Use Program of Nintedanib for the Treatment of Idiopathic Pulmonary Fibrosis. [2017]

4. Iran pubmed.ncbi.nlm.nih.gov

Efficacy and Safety of Nintedanib in Patients with Interstitial Lung Disease with or without Systemic Sclerosis: A Meta-Analysis. [2023]

5. England pubmed.ncbi.nlm.nih.gov

Real-life efficacy and safety of nintedanib in systemic sclerosis-interstitial lung disease: data from an Italian multicentre study. [2023]

6. Switzerland 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. [2023]

7. United States pubmed.ncbi.nlm.nih.gov

Safety of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis: Global Pharmacovigilance Data. [2021]

8. United States 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. [2023]

9. Italy pubmed.ncbi.nlm.nih.gov

Association of low body surface area with dose reduction and/or discontinuation of nintedanib in patients with idiopathic pulmonary fibrosis: a pilot study. [2022]

10. England pubmed.ncbi.nlm.nih.gov

Safety and tolerability of nintedanib in patients with systemic sclerosis-associated interstitial lung disease: data from the SENSCIS trial. [2022]

11. New Zealand pubmed.ncbi.nlm.nih.gov

A high bioavailability and sustained-release nano-delivery system for nintedanib based on electrospray technology. [2023]

12. United States pubmed.ncbi.nlm.nih.gov

Nintedanib: a novel therapeutic approach for idiopathic pulmonary fibrosis. [2014]

13. England pubmed.ncbi.nlm.nih.gov

Nintedanib for the treatment of systemic sclerosis-associated interstitial lung disease. [2021]