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

NCT05285982 | Completed Phase 3 Results DMC FDA Drug

• 4 other identifiers: 1199-03782020-005554-23U1111-1305-75142024-515743-27-00
• Study Type: interventional
• Target: 54
• Locations: 17 countries
• Sites: 28

Brief 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 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

Outcome Measures

Primary Outcomes (1)

• 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.

  From first drug administration until end of residual effect period (REP) 28 days after the last dose of trial medication, up to 1127 days.

Study Arms (1)

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).

Drug: Nintedanib (Ofev®)

Interventions

Nintedanib (Ofev®) DRUG

Nintedanib soft capsules administered orally with doses ranged from 50 to 150 mg bid depending on body weight

Also known as: Ofev

Nintedanib

Eligibility Criteria

• Age: 6 Years - 17 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17)

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:
• Patients who completed the InPedILD® trial as planned and who did not permanently prematurely discontinue study treatment.

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.

Sponsors & Collaborators

• Boehringer Ingelheim: lead

Study Sites (28)

Children's Hospital Los Angeles

Los Angeles, California, 90027, United States

Location

Children's Hospital Colorado

Aurora, Colorado, 80045, United States

Location

Weill Cornell Medicine-New York-60569

New York, New York, 10021, United States

Location

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, 19104, United States

Location

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

Pittsburgh, Pennsylvania, 15224, United States

Location

Vanderbilt University Medical Center

Nashville, Tennessee, 37232, United States

Location

Hospital de Pediatria Prof. Dr. Juan P. Garrahan

CABA, C1245AAM, Argentina

Location

Hospital de Niños Dr. Ricardo Gutierrez

CABA, C1425EFD, Argentina

Location

Brussels - UNIV HUDERF

Brussels, 1020, Belgium

Location

Serviços Medicos Respirar Sul Fluminense

Barra Mansa, 27323240, Brazil

Location

Centro de Pesquisa Clinica do Instituto da Crianca - HCFMUSP

São Paulo, 5403-900, Brazil

Location

BC Children's Hospital

Vancouver, British Columbia, V6H 3N1, Canada

Location

The Hospital for Sick Children

Toronto, Ontario, M5G 1X8, Canada

Location

Teaching Hospital Motol, Oncology Clinic

Prague, 150 06, Czechia

Location

Tampere University Hospital

Tampere, 33520, Finland

Location

HOP Intercommunal

Créteil, 94010, France

Location

Hamburger Zentrum für Kinder- und Jugendrheumatologie

Hamburg, 22081, Germany

Location

General Hospital of Thessaloniki "Ippokrateio"

Thessaloniki, 54642, Greece

Location

Azienda Ospedaliera Meyer

Florence, 50139, Italy

Location

Osp. Pediatrico Bambin Gesù

Roma, 00165, Italy

Location

Clinical Research Institute S.C.

Tlalnepantla, 54055, Mexico

Location

Oslo Universitetssykehus HF, Rikshospitalet

Oslo, N-0372, Norway

Location

Independent Public Teaching Children's Hospital

Warsaw, 02091, Poland

Location

ULS de São José, E.P.E. - Hospital Dona Estefânia

Lisbon, 1169-045, Portugal

Location

ULS de Santa Maria, E.P.E

Lisbon, 1649-035, Portugal

Location

Hospital Universitari Vall D Hebron

Barcelona, 08035, Spain

Location

Hospital Virgen del Rocío

Seville, 41013, Spain

Location

King's College Hospital

London, SE5 9RS, United Kingdom

Location

Related Links

• Related Info

MeSH Terms

Conditions

Lung Diseases, Interstitial

Interventions

nintedanib

Condition Hierarchy (Ancestors)

Lung Diseases; Respiratory Tract Diseases

Limitations and Caveats

Some limitations due to the nature of the extension trial should be considered when interpreting the data (i.e. bias in the selection of the population and no comparative arm). All endpoints were considered exploratory only.

Results Point of Contact

• Title: Boehringer Ingelheim, Call Center
• Organization: Boehringer Ingelheim

clintriage.rdg@boehringer-ingelheim.com

018002430127

Publication Agreements

• PI is Sponsor Employee: No
• Restriction Type: OTHER
• Restrictive Agreement: Yes

Study Design

• Study Type: interventional
• Phase: phase 3
• Allocation: NA
• Masking: NONE
• Purpose: TREATMENT
• Intervention Model: SINGLE GROUP
• Sponsor Type: INDUSTRY
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: March 10, 2022
• First Posted: March 18, 2022
• Study Start: April 6, 2022
• Primary Completion: August 13, 2025
• Study Completion: August 13, 2025
• Last Updated: March 4, 2026
• Results First Posted: March 4, 2026

Record last verified: 2026-01

Data Sharing

• IPD Sharing: Will share
• Shared Documents: STUDY PROTOCOL, SAP, CSR
• Time Frame: After structured results have been posted, all regulatory activities are completed in the US and EU for the product and indication, and after the primary manuscript has been accepted for publication.
• Access Criteria: For study documents - upon signing of a 'Document Sharing Agreement'. For study data - 1. after the submission and approval of the research proposal (checks will be performed by the sponsor and/or the independent review panel, including checking that the planned analysis does not compete with sponsor's publication plan); 2. and upon signing of a legal agreement.

Locations

PL (1); BE (1); FR (1); BR (2); PT (2); DE (1); IT (2); ES (2); CA (2); NO (1); US (6); CZ (1); GB (1); ME (1); AR (2); FI (1); GR (1)