Lung Diseases, InterstitialPhase 3NCT05285982
A Study to Evaluate Long-term Safety of Nintedanib in Children and Adolescents With Interstitial Lung Disease (InPedILD®-ON)
A Phase 3 study testing Nintedanib (Ofev®) in people with Lung Diseases, Interstitial, run by Boehringer Ingelheim at 28 sites in 17 countries.
Trial summary
This study is open to children and adolescents with interstitial lung disease (ILD) that causes lung fibrosis. This is a study for people who took part in a previous study called InPedILD (study 1199-0337) and for people who are between 6 and 17 years old (in France, between 12 and 17 years old) and have fibrosing… Read more about this study
- Who is it for?
- Ages 6 to 17 years
- Conditions the study is for
- Lung Diseases, Interstitial
- What is being tested?
- Nintedanib (Ofev®)
- Type of treatment
- Pill or oral medicine
- Could I receive a placebo?
- No, everyone receives an active treatment
- How many people
- 54 participants
- Where
- 28 sitesin 17 countries
- Study dates
- 6 Apr 2022to 13 Aug 2025
- Phase
- Phase 3
- Are there results?
- Yes, posted in the registrySummary in the doctors' view
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 6 to 17 years
- All sexes
- No healthy volunteers
- Child
Who may be able to join
- For new patients:
- Children and adolescents 6 to 17 years old at Visit 2. In France, only adolescents 12 to 17 years old at Visit 2.
- Signed and dated written informed consent and assent, where applicable, in accordance with ICH-GCP and local legislation prior to admission to the trial.
- Male or female patients. Female of childbearing potential (WOCBP1) must confirm that sexual abstinence is standard practice and will be continued until 3 months after last drug intake, or be ready and able to use a highly effective method of birth control per ICH M3 (R2) that results in a low failure rate of less than 1% per year when used consistently and correctly, in combination with one barrier method, from 28 days prior to initiation of study treatment, during treatment and until 3 months after last drug intake. Sexual abstinence is defined as abstinence from any sexual act that may result in pregnancy.
- Patients with evidence of fibrosing Interstitial Lung Disease (ILD) on High-Resolution Computed Tomography (HRCT) within 12 months of Visit 1 as assessed by the investigator and confirmed by central review.
- Patients with Forced Vital Capacity (FVC) % predicted ≥25% at Visit 2.
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- Patients with clinically significant disease at Visit 2, as assessed by the investigator based on any of the following:
- Fan score ≥3, or
- Documented evidence of clinical progression over time based on either
- a 5-10% relative decline in FVC % predicted accompanied by worsening symptoms, or
- a ≥10% relative decline in FVC % predicted, or
- increased fibrosis on HRCT, or
- other measures of clinical worsening attributed to progressive lung disease (e.g. increased oxygen requirement, decreased diffusion capacity).
- For roll-over patients from the InPedILD® study:
- Only criteria 2 and 3 listed for new patients are applicable with the following additional inclusion criterion:
- Patients who completed the InPedILD® trial as planned and who did not permanently prematurely discontinue study treatment.
- For patients who prematurely discontinued treatment permanently in 1199-0337 but are potentially eligible and for completed patients from parent trial not able to roll over into the extension trial within 12 weeks following their End of Treatment Visit in the parent trial:
- Inclusion criteria for new patients are applicable except criteria 4, and 6 (as eligibility for these criteria has been confirmed already in 1199-0337 and does not need to be repeated) and also except inclusion criterion 1 for completed patients from parent trial not able to roll over within 12 weeks following their End of Treatment Visit in the parent trial.
Who cannot join
- For new patients:
- Aspartate Aminotransferase (AST) and/or Alanine Aminotransferase (ALT) >1.5 x Upper limit of normal (ULN) at Visit 1.
- Bilirubin >1.5 x ULN at Visit 1.
- Estimated Glomerular Filtration Rate (eGFR) <30 mL/min/1.73 m² at Visit 1
- Patients with underlying chronic liver disease (Child Pugh A, B or C hepatic impairment) at Visit 1.
- Other investigational therapy received within 1 month or 5 half-lives (whichever is shorter but ≥1 week) prior to Visit 2 except investigational therapy received in InPedILD® trial.
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- Significant pulmonary arterial hypertension (PAH) defined by any of the following:
- Previous clinical or echocardiographic evidence of significant right heart failure
- History of right heart catheterization showing a cardiac index ≤2 l/min/m²
- PAH requiring parenteral therapy with epoprostenol/treprostinil
- In the opinion of the Investigator, other clinically significant pulmonary abnormalities.
- Cardiovascular diseases, any of the following:
- Severe hypertension, uncontrolled under treatment, within 6 months of Visit 1. Uncontrolled hypertension is defined as
- In children 6 to ≤12 years old: ≥95th percentile + 12 mm Hg or ≥140/90 mm Hg (whichever is lower) (systolic or diastolic blood pressure equal to or greater than the calculated target value). Not applicable in France.
- In adolescents 13 to 17 years old: systolic blood pressure ≥140 mm Hg or diastolic blood pressure ≥90 mm Hg. Not applicable in France.
- Myocardial infarction within 6 months of Visit 1
- Unstable cardiac angina within 6 months of Visit 1
- Bleeding risk, any of the following:
- Known genetic predisposition to bleeding
- Patients who require
- Fibrinolysis, full-dose therapeutic anticoagulation (e.g. vitamin K antagonists, direct thrombin inhibitors, heparin, hirudin)
- High dose antiplatelet therapy
- History of haemorrhagic central nervous system (CNS) event within 12 months of Visit 1
- Any of the following within 3 months of Visit 1:
- Haemoptysis or haematuria
- Active gastro-intestinal (GI) bleeding or GI - ulcers
- Major injury or surgery (investigator's judgment)
- Any of the following coagulation parameters at Visit 1:
- International normalized ratio (INR) >2
- Prolongation of prothrombin time (PT) by >1.5 x ULN
- Prolongation of activated partial thromboplastin time (aPTT) by >1.5 x ULN
- History of thrombotic event (including stroke and transient ischemic attack) within 12 months of Visit 1.
- Known hypersensitivity to the trial medication or its components (i.e. soya lecithin).
- Patients with documented allergy to peanut or soya.
- Other disease that may interfere with testing procedures or in the judgment of the investigator may interfere with trial participation or may put the patient at risk when participating in this trial.
- Life expectancy for any concomitant disease other than ILD <2.5 years (investigator assessment).
- Female patients who are pregnant, nursing, or who plan to become pregnant while in the trial.
- Patients not able or willing to adhere to trial procedures, including intake of study medication.
- Patients who must or wish to take any drug considered likely to interfere with the safe conduct of the trial according to investigator's benefit-risk assessment for the individual patient
- Patients with any diagnosed growth disorder such as growth hormone deficiency or any genetic disorder that is associated with short stature (e.g. Turner Syndrome, Noonan Syndrome, Russell-Silver Syndrome) and/or treatment with growth hormone therapy within 6 months before Visit 2. Patients with short stature considered by the investigator to be due to glucocorticoid therapy may be included.
- Patients <13.5 kg of weight at Visit 1 (same threshold to be used for male and female patients).
- For roll-over patients from the InPedILD® study:
- Only criteria 11, 12, 13, 15, 16, 17 and 19, listed for new patients are applicable with the following additional exclusion criterion:
- Patient not compliant in parent trial (InPedILD®), with trial medication or trial visits, according to investigator's judgement. Roll-over patients may qualify for participation even though other exclusion criteria may have been met during the participation in InPedILD®, if the investigator's benefit-risk assessment for the individual patient remains favorable.
- For patients who prematurely discontinued treatment permanently in 1199-0337 but are potentially eligible and for completed patients from parent trial not able to roll over into the extension trial within 12 weeks following their End of Treatment Visit in the parent trial:
- All exclusion criteria for new patients are applicable. In addition, the following additional exclusion criterion is applicable for patients who prematurely discontinued treatment permanently in 1199-0337:
- Patients who experienced drug-related adverse events during parent trial leading to permanent study treatment discontinuation.
Trial locations and contacts
28 sites in 17 countries.
28 sites in 17 countries
United States6 sites
Children's Hospital Colorado
Aurora, Colorado, 80045
Children's Hospital Los Angeles
Los Angeles, California, 90027
Vanderbilt University Medical Center
Nashville, Tennessee, 37232
Weill Cornell Medicine-New York-60569
New York, New York, 10021
Children's Hospital of Philadelphia
Philadelphia, Pennsylvania, 19104
Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center
Pittsburgh, Pennsylvania, 15224
Argentina2 sites
Hospital de Pediatria Prof. Dr. Juan P. Garrahan
CABA, C1245AAM
Hospital de Niños Dr. Ricardo Gutierrez
CABA, C1425EFD
Brazil2 sites
Serviços Medicos Respirar Sul Fluminense
Barra Mansa, 27323240
Centro de Pesquisa Clinica do Instituto da Crianca - HCFMUSP
São Paulo, 5403-900
Canada2 sites
The Hospital for Sick Children
Toronto, Ontario, M5G 1X8
BC Children's Hospital
Vancouver, British Columbia, V6H 3N1
Italy2 sites
Azienda Ospedaliera Meyer
Florence, 50139
Osp. Pediatrico Bambin Gesù
Roma, 00165
Portugal2 sites
ULS de São José, E.P.E. - Hospital Dona Estefânia
Lisbon, 1169-045
ULS de Santa Maria, E.P.E
Lisbon, 1649-035
Spain2 sites
Hospital Universitari Vall D Hebron
Barcelona, 08035
Hospital Virgen del Rocío
Seville, 41013
Germany1 site
Hamburger Zentrum für Kinder- und Jugendrheumatologie
Hamburg, 22081
Greece1 site
General Hospital of Thessaloniki "Ippokrateio"
Thessaloniki, 54642
About this study
Official title: An Open-label Trial of the Long-term Safety and Tolerability of Nintedanib Per os, on Top of Standard of Care, Over at Least 3 Years, in Children and Adolescents With Clinically Significant Fibrosing Interstitial Lung Disease (InPedILD®-ON)
This study is open to children and adolescents with interstitial lung disease (ILD) that causes lung fibrosis. This is a study for people who took part in a previous study called InPedILD (study 1199-0337) and for people who are between 6 and 17 years old (in France, between 12 and 17 years old) and have fibrosing ILD.
This study tests a medicine called nintedanib. Nintedanib is already used to treat different types of lung fibrosis in adults. The purpose of the study is to find out how well long-term treatment with nintedanib is tolerated in children and adolescents.
All participants take nintedanib capsules twice a day. Participants coming from the previous study are in this study for at least 3 years or until nintedanib or other treatment options become available outside of this study. New participants are in the study until the overall end of study meaning for at least 1.5 years. Participants visit the study site about 15 times for a study participation of 3 years. Afterwards, they visit the study site every 3 months. The doctors collect information on any health problems of the participants.
- Conditions
- Lung Diseases, Interstitial
- Being tested
- Nintedanib (Ofev®)
- Medical subject headings
- Lung Diseases, Interstitial
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
Nintedanib (Ofev®)Drug
Also called Ofev
Nintedanib soft capsules administered orally with doses ranged from 50 to 150 mg bid depending on body weight
| Study group | Role | What they receive |
|---|---|---|
| Nintedanib | Experimental | Pediatric patients with clinically significant fibrosing intestitial lung disease (ILD) who either completed the parent trial 1199-0337 or were new patients received nintedanib soft gelatine capsules. Nintedanib doses ranged from 50 milligrams (mg) bid (13.5 to < 23 kilograms (kg) bodyweight) to 150 mg bid (>=57.5 kg bodyweight). Receives: Drug: Nintedanib (Ofev®) |
What is being measured
The primary outcome is the main question the study is designed to answer.
Number of Patients With Treatment-emergent Adverse Events (AEs) Over the Whole Trial
Number of patients with treatment-emergent adverse events (AEs) over the whole trial.
Measured: From first drug administration until end of residual effect period (REP) 28 days after the last dose of trial medication, up to 1127 days.
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted18 Mar 2022
- Study start6 Apr 2022
- Primary completion13 Aug 2025
- Study completion13 Aug 2025
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Posted resultsParticipant flow, outcome measures and adverse events reported by the sponsor.
- Other trials for Lung Diseases, InterstitialStudies of the same condition, open and under way.
- More trials run by Boehringer IngelheimThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Interventional
- Phase
- Phase 3
- Primary purpose
- Treatment
- Allocation
- Not applicable
- Intervention model
- Single group
- Masking
- None (open label)
- Enrolment
- 54 participants (actual)
Sponsor and investigators
- Lead sponsor
- Boehringer Ingelheim
- Sponsor type
- Industry (pharma, biotech, devices)
- Responsible party
- Sponsor
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT05285982
- Sponsor's protocol ID
- 1199-0378
- Other IDs
- 2020-005554-23, U1111-1305-7514, 2024-515743-27-00
- Status last verified
- Jan 2026
- First submitted
- 10 Mar 2022
- Last updated
- 4 Mar 2026
- Results posted
- Yes, on ClinicalTrials.gov
- Data monitoring committee
- Yes
- FDA-regulated drug
- Yes
- FDA-regulated device
- No
- Individual data sharing
- Yes: Once the criteria in section "Time Frame" are fulfilled, researchers can use the following link https://www.mystudywindow.com/msw/datasharing to request access to the clinical study documents regarding this study, and upon a signed "Document Sharing Agreement". Furthermore, researchers can request access to the clinical study data, for this and other listed studies, after the submission of a research proposal and according to the terms outlined in the website.
Study at a glance
From the registry record, last updated by the sponsor on 4 Mar 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- An Open-label Trial of the Long-term Safety and Tolerability of Nintedanib Per os, on Top of Standard of Care, Over at Least 3 Years, in Children and Adolescents With Clinically Significant Fibrosing Interstitial Lung Disease (InPedILD®-ON)
- ClinicalTrials.gov ID
- NCT05285982
- Acronym / sponsor ID
- 1199-0378
- Conditions
- Lung Diseases, Interstitial
- Phase
- Phase 3
- Study type
- Interventional
- Recruitment status
- Completed
- Enrolment
- 54 participants (actual)
- Lead sponsor
- Boehringer IngelheimIndustry (pharma, biotech, devices)
- Study start
- 6 Apr 2022 (actual)
- Primary completion
- 13 Aug 2025 (actual)
- Study completion
- 13 Aug 2025 (actual)
- First posted
- 18 Mar 2022 (actual)
- Results
- Posted 4 Mar 2026
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
This study is open to children and adolescents with interstitial lung disease (ILD) that causes lung fibrosis. This is a study for people who took part in a previous study called InPedILD (study 1199-0337) and for people who are between 6 and 17 years old (in France, between 12 and 17 years old) and have fibrosing ILD.
This study tests a medicine called nintedanib. Nintedanib is already used to treat different types of lung fibrosis in adults. The purpose of the study is to find out how well long-term treatment with nintedanib is tolerated in children and adolescents.
All participants take nintedanib capsules twice a day. Participants coming from the previous study are in this study for at least 3 years or until nintedanib or other treatment options become available outside of this study. New participants are in the study until the overall end of study meaning for at least 1.5 years. Participants visit the study site about 15 times for a study participation of 3 years. Afterwards, they visit the study site every 3 months. The doctors collect information on any health problems of the participants.
Study design
- Primary purpose
- Treatment
- Allocation
- Not applicable
- Intervention model
- Single group
- Masking
- None (open label)
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| Nintedanib | Experimental | Drug: Nintedanib (Ofev®)Pediatric patients with clinically significant fibrosing intestitial lung disease (ILD) who either completed the parent trial 1199-0337 or were new patients received nintedanib soft gelatine capsules. Nintedanib doses ranged from 50 milligrams (mg) bid (13.5 to < 23 kilograms (kg) bodyweight) to 150 mg bid (>=57.5 kg bodyweight). |
Who may qualify
- Ages
- 6 to 17 years
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Child
Key inclusion criteria (18 listed)
- Children and adolescents 6 to 17 years old at Visit 2. In France, only adolescents 12 to 17 years old at Visit 2.
- Signed and dated written informed consent and assent, where applicable, in accordance with ICH-GCP and local legislation prior to admission to the trial.
- Male or female patients. Female of childbearing potential (WOCBP1) must confirm that sexual abstinence is standard practice and will be continued until 3 months after last drug intake, or be ready and able to use a highly effective method of birth control per ICH M3 (R2) that results in a low failure rate of less than 1% per year when used consistently and correctly, in combination with one barrier method, from 28 days prior to initiation of study treatment, during treatment and until 3 months after last drug intake. Sexual abstinence is defined as abstinence from any sexual act that may result in pregnancy.
- Patients with evidence of fibrosing Interstitial Lung Disease (ILD) on High-Resolution Computed Tomography (HRCT) within 12 months of Visit 1 as assessed by the investigator and confirmed by central review.
- Patients with Forced Vital Capacity (FVC) % predicted ≥25% at Visit 2.
Key exclusion criteria (47 listed)
- Aspartate Aminotransferase (AST) and/or Alanine Aminotransferase (ALT) >1.5 x Upper limit of normal (ULN) at Visit 1.
- Bilirubin >1.5 x ULN at Visit 1.
- Estimated Glomerular Filtration Rate (eGFR) <30 mL/min/1.73 m² at Visit 1
- Patients with underlying chronic liver disease (Child Pugh A, B or C hepatic impairment) at Visit 1.
- Other investigational therapy received within 1 month or 5 half-lives (whichever is shorter but ≥1 week) prior to Visit 2 except investigational therapy received in InPedILD® trial.
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| Nintedanib (Ofev®)Also: OfevNintedanib soft capsules administered orally with doses ranged from 50 to 150 mg bid depending on body weight | Drug | Nintedanib |
Primary outcome
Number of Patients With Treatment-emergent Adverse Events (AEs) Over the Whole Trial
Time frame: From first drug administration until end of residual effect period (REP) 28 days after the last dose of trial medication, up to 1127 days.
Number of patients with treatment-emergent adverse events (AEs) over the whole trial.
Results and safety
Loading the posted results from ClinicalTrials.gov…
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 4 Mar 2026.
- Posted resultsParticipant flow, baseline, all outcome measures and adverse events.
- Related InfoLink provided by the sponsor.
- Individual participant dataSharing planned: Yes. Once the criteria in section "Time Frame" are fulfilled, researchers can use the following link https://www.mystudywindow.com/msw/datasharing to request access to the clinical study documents regarding this study, and upon a signed "Document Sharing Agreement". Furthermore, researchers can request access to the clinical study data, for this and other listed studies, after the submission of a research proposal and according to the terms outlined in the website.
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
The record lists no contact. Use the official record to reach the sponsor.
28 sites in 17 countries: see every site and its status.
Questions about this trial
What is this clinical trial studying?
A Phase 3 study testing Nintedanib (Ofev®) in people with Lung Diseases, Interstitial, run by Boehringer Ingelheim at 28 sites in 17 countries.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 18 inclusion and 47 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: finished, as of the sponsor's last update on 4 Mar 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 28 sites in United States, Argentina, Brazil, Canada, Italy and 12 more countries. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
What does this phase mean?
Phase 3 studies compare the treatment with the current standard treatment or a placebo in a large group, to confirm how well it works and watch for side effects.
Not every study follows the same path, and some studies (observational studies, device studies) have no phase.
Are results available for this study?
Yes. Results are posted in the registry (first posted 4 Mar 2026). A summary is in the For doctors view, and the full results are on ClinicalTrials.gov.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Number of Patients With Treatment-emergent Adverse Events (AEs) Over the Whole Trial (time frame: From first drug administration until end of residual effect period (REP) 28 days after the last dose of trial medication, up to 1127 days.). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 4 Mar 2026.