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A Study to Evaluate Long-term Safety of Nintedanib in Children and Adolescents With Interstitial Lung Disease (InPedILD®-ON)

Study Purpose

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 (study 1199-0337, InPedILD™) and for people who are between 6 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 are in the study for at least 6 months or until nintedanib or other treatment options become available outside of this study. During the first 2 years, they visit the study site between 9 and 11 times. Afterwards, they visit the study site every 3 months. The doctors collect information on any health problems of the participants.

Recruitment Criteria

Accepts Healthy Volunteers

Healthy volunteers are participants who do not have a disease or condition, or related conditions or symptoms

No
Study Type

An interventional clinical study is where participants are assigned to receive one or more interventions (or no intervention) so that researchers can evaluate the effects of the interventions on biomedical or health-related outcomes.


An observational clinical study is where participants identified as belonging to study groups are assessed for biomedical or health outcomes.


Searching Both is inclusive of interventional and observational studies.

Interventional
Eligible Ages 6 Years - 17 Years
Gender All
More Inclusion & Exclusion Criteria

Inclusion Criteria:

For new patients: 1. Children and adolescents 6 to 17 years old at Visit 2. 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. 3. 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. 4. 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. 5. Patients with Forced Vital Capacity (FVC) % predicted ≥25% at Visit 2. 6. 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: 7. 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.

Exclusion Criteria:

For new patients: 1. Aspartate Aminotransferase (AST) and/or Alanine Aminotransferase (ALT) >1.5 x Upper limit of normal (ULN) at Visit 1. 2. Bilirubin >1.5 x ULN at Visit 1. 3. Estimated Glomerular Filtration Rate (eGFR) <30 mL/min/1.73 m² at Visit 1. 4. Patients with underlying chronic liver disease (Child Pugh A, B or C hepatic impairment) at Visit 1. 5. 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. 6. 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.
7. In the opinion of the Investigator, other clinically significant pulmonary abnormalities. 8. 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) - In adolescents 13 to 17 years old: systolic blood pressure ≥140 mm Hg or diastolic blood pressure ≥90 mm Hg.
  • - Myocardial infarction within 6 months of Visit 1.
  • - Unstable cardiac angina within 6 months of Visit 1.
9. 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.
10. History of thrombotic event (including stroke and transient ischemic attack) within 12 months of Visit 1. 11. Known hypersensitivity to the trial medication or its components (i.e. soya lecithin). 12. Patients with documented allergy to peanut or soya. 13. 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. 14. Life expectancy for any concomitant disease other than ILD <2.5 years (investigator assessment). 15. Female patients who are pregnant, nursing, or who plan to become pregnant while in the trial. 16. Patients not able or willing to adhere to trial procedures, including intake of study medication. 17. 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. 18. 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. 19. 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: 20. 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: 21. Patients who experienced drug-related adverse events during parent trial leading to permanent study treatment discontinuation.

Trial Details

Trial ID:

This trial id was obtained from ClinicalTrials.gov, a service of the U.S. National Institutes of Health, providing information on publicly and privately supported clinical studies of human participants with locations in all 50 States and in 196 countries.

NCT05285982
Phase

Phase 1: Studies that emphasize safety and how the drug is metabolized and excreted in humans.

Phase 2: Studies that gather preliminary data on effectiveness (whether the drug works in people who have a certain disease or condition) and additional safety data.

Phase 3: Studies that gather more information about safety and effectiveness by studying different populations and different dosages and by using the drug in combination with other drugs.

Phase 4: Studies occurring after FDA has approved a drug for marketing, efficacy, or optimal use.

Phase 3
Lead Sponsor

The sponsor is the organization or person who oversees the clinical study and is responsible for analyzing the study data.

Boehringer Ingelheim
Principal Investigator

The person who is responsible for the scientific and technical direction of the entire clinical study.

N/A
Principal Investigator Affiliation N/A
Agency Class

Category of organization(s) involved as sponsor (and collaborator) supporting the trial.

Industry
Overall Status Recruiting
Countries Argentina, Belgium, Brazil, Canada, Czechia, Finland, France, Germany, Greece, Italy, Mexico, Norway, Poland, Portugal, Spain, United Kingdom, United States
Conditions

The disease, disorder, syndrome, illness, or injury that is being studied.

Lung Diseases, Interstitial
Study Website: View Trial Website
Arms & Interventions

Arms

Experimental: Patients rolling over from the InPedILD® study

Experimental: Patients newly enrolled in this study

Interventions

Drug: - Nintedanib (Ofev®)

Nintedanib (Ofev®) soft capsules

Contact a Trial Team

If you are interested in learning more about this trial, find the trial site nearest to your location and contact the site coordinator via email or phone. We also strongly recommend that you consult with your healthcare provider about the trials that may interest you and refer to our terms of service below.

Children's Hospital Los Angeles, Los Angeles, California

Status

Recruiting

Address

Children's Hospital Los Angeles

Los Angeles, California, 90027

Site Contact

Boehringer Ingelheim

unitedstates@bitrialsupport.com

833-602-2368

Children's Hospital Colorado, Aurora, Colorado

Status

Active, not recruiting

Address

Children's Hospital Colorado

Aurora, Colorado, 80045

Weill Cornell Medicine, New York, New York

Status

Active, not recruiting

Address

Weill Cornell Medicine

New York, New York, 10021

Children's Hospital of Philadelphia, Philadelphia, Pennsylvania

Status

Active, not recruiting

Address

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, 19104

Pittsburgh, Pennsylvania

Status

Completed

Address

Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center

Pittsburgh, Pennsylvania, 15224

Vanderbilt University Medical Center, Nashville, Tennessee

Status

Active, not recruiting

Address

Vanderbilt University Medical Center

Nashville, Tennessee, 37232

International Sites

Caba, Argentina

Status

Recruiting

Address

Hospital de Pediatría " Prof. Dr. Juan P. Garrahan"

Caba, , C1245AAM

Site Contact

Boehringer Ingelheim

argentina@bitrialsupport.com

08002667801

Hospital de Niños Dr. Ricardo Gutierrez, Caba, Argentina

Status

Recruiting

Address

Hospital de Niños Dr. Ricardo Gutierrez

Caba, , C1425EFD

Site Contact

Boehringer Ingelheim

argentina@bitrialsupport.com

08002667801

Brussels - UNIV HUDERF, Bruxelles, Belgium

Status

Active, not recruiting

Address

Brussels - UNIV HUDERF

Bruxelles, , 1020

Serviços Medicos Respirar Sul Fluminense, Barra Mansa, Brazil

Status

Recruiting

Address

Serviços Medicos Respirar Sul Fluminense

Barra Mansa, , 27323240

Site Contact

Boehringer Ingelheim

brasil@bitrialsupport.com

08008919295

Sao Paulo, Brazil

Status

Recruiting

Address

Centro de Pesquisa Clinica do Instituto da Crianca - HCFMUSP

Sao Paulo, , 5403-900

Site Contact

Boehringer Ingelheim

brasil@bitrialsupport.com

08008919295

BC Children's Hospital, Vancouver, British Columbia, Canada

Status

Active, not recruiting

Address

BC Children's Hospital

Vancouver, British Columbia, V6H 3N1

The Hospital for Sick Children, Toronto, Ontario, Canada

Status

Active, not recruiting

Address

The Hospital for Sick Children

Toronto, Ontario, M5G 1X8

Teaching Hospital Motol, Oncology Clinic, Praha 5, Czechia

Status

Recruiting

Address

Teaching Hospital Motol, Oncology Clinic

Praha 5, , 150 06

Site Contact

Boehringer Ingelheim

cesko@bitrialsupport.com

800142046

Tampere University Hospital, Tampere, Finland

Status

Terminated

Address

Tampere University Hospital

Tampere, , 33520

HOP Intercommunal, Créteil, France

Status

Recruiting

Address

HOP Intercommunal

Créteil, , 94000

Site Contact

Boehringer Ingelheim

france@bitrialsupport.com

0805102354

Hamburg, Germany

Status

Recruiting

Address

Hamburger Zentrum für Kinder- und Jugendrheumatologie

Hamburg, , 22081

Site Contact

Boehringer Ingelheim

deutschland@bitrialsupport.com

08007234742

Thessaloniki, Greece

Status

Active, not recruiting

Address

General Hospital of Thessaloniki "Ippokrateio"

Thessaloniki, , 54642

Azienda Ospedaliera Meyer, Firenze, Italy

Status

Recruiting

Address

Azienda Ospedaliera Meyer

Firenze, , 50139

Site Contact

Boehringer Ingelheim

italia@bitrialsupport.com

800977373

Osp. Pediatrico Bambin Gesù, Roma, Italy

Status

Recruiting

Address

Osp. Pediatrico Bambin Gesù

Roma, , 00165

Site Contact

Boehringer Ingelheim

italia@bitrialsupport.com

800977373

Clinical Research Institute S.C., Tlalnepantla, Mexico

Status

Recruiting

Address

Clinical Research Institute S.C.

Tlalnepantla, , 54055

Site Contact

Boehringer Ingelheim

mexico@bitrialsupport.com

018000623749

Oslo, Norway

Status

Active, not recruiting

Address

Oslo Universitetssykehus HF, Rikshospitalet

Oslo, , N-0372

Warsaw, Poland

Status

Recruiting

Address

Independent Public Teaching Children's Hospital

Warsaw, , 02091

Site Contact

Boehringer Ingelheim

polska@bitrialsupport.com

008001218830

CHULC, EPE - Hospital Dona Estefânia, Lisboa, Portugal

Status

Active, not recruiting

Address

CHULC, EPE - Hospital Dona Estefânia

Lisboa, , 1169-045

Lisboa, Portugal

Status

Active, not recruiting

Address

Centro Hospitalar Universitário de Lisboa Norte, EPE - Hospital Santa Maria

Lisboa, , 1649-035

Hospital Vall d'Hebron, Barcelona, Spain

Status

Recruiting

Address

Hospital Vall d'Hebron

Barcelona, , 08035

Site Contact

Boehringer Ingelheim

espana@bitrialsupport.com

900876092

Hospital Virgen del Rocío, Sevilla, Spain

Status

Recruiting

Address

Hospital Virgen del Rocío

Sevilla, , 41013

Site Contact

Boehringer Ingelheim

espana@bitrialsupport.com

900876092

King's College Hospital, London, United Kingdom

Status

Completed

Address

King's College Hospital

London, , SE5 9RS