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

Completed · Phase 3

Conditions studied: Lung Diseases, Interstitial

In brief

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.

Key facts

Study ID: NCT05285982
Run by: Boehringer Ingelheim
People needed: 54
Starts: 2022-04-06
Expected to finish: 2025-08-13
Last updated by the study team: 2026-03-04

Who can join

Age: 6 and older, up to 17. Sex: any. Healthy volunteers: not accepted.

You may qualify if…

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.
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.

You may not qualify if…

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.
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

Where it is running

Children's Hospital Los Angeles — Los Angeles, California, United States
Children's Hospital Colorado — Aurora, Colorado, United States
Weill Cornell Medicine-New York-60569 — New York, New York, United States
Children's Hospital of Philadelphia — Philadelphia, Pennsylvania, United States
Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center — Pittsburgh, Pennsylvania, United States
Vanderbilt University Medical Center — Nashville, Tennessee, United States
Hospital de Pediatria Prof. Dr. Juan P. Garrahan — CABA, Argentina
Hospital de Niños Dr. Ricardo Gutierrez — CABA, Argentina
Brussels - UNIV HUDERF — Brussels, Belgium
Serviços Medicos Respirar Sul Fluminense — Barra Mansa, Brazil
Centro de Pesquisa Clinica do Instituto da Crianca - HCFMUSP — São Paulo, Brazil
BC Children's Hospital — Vancouver, British Columbia, Canada
The Hospital for Sick Children — Toronto, Ontario, Canada
Teaching Hospital Motol, Oncology Clinic — Prague, Czechia
Tampere University Hospital — Tampere, Finland
HOP Intercommunal — Créteil, France
Hamburger Zentrum für Kinder- und Jugendrheumatologie — Hamburg, Germany
General Hospital of Thessaloniki "Ippokrateio" — Thessaloniki, Greece
Azienda Ospedaliera Meyer — Florence, Italy
Osp. Pediatrico Bambin Gesù — Roma, Italy
Clinical Research Institute S.C. — Tlalnepantla, Mexico
Oslo Universitetssykehus HF, Rikshospitalet — Oslo, Norway
Independent Public Teaching Children's Hospital — Warsaw, Poland
ULS de São José, E.P.E. - Hospital Dona Estefânia — Lisbon, Portugal
ULS de Santa Maria, E.P.E — Lisbon, Portugal

Full record on ClinicalTrials.gov

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