Interstitial Lung Disease MedDRA version: 21.1 Level: LLT Classification code 10066393 Term: Respiratory bronchiolitis-associated interstitial lung disease System Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders
Conditions
Sponsors
Boehringer Ingelheim SComm
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: For new patients: 1. Children and adolescents 6 to 17 years old at Visit 2. In France, only adolescents 12 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 ILD on HRCT within 12 months of Visit 1 as assessed by the investigator and confirmed by central review. 5. Patients with 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 o a 5-10% relative decline in FVC% predicted accompanied by worsening symptoms, or o a =10% relative decline in FVC % predicted, or o increased fibrosis on HRCT, or o other measures of clinical worsening attributed to progressive lung disease 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 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: Criteria for new patients are applicable except criteria 4, and 6 and also except inclusion criterion 1 for completed patients from parent trial not able to roll-over into the extension trial within 12 weeks following their end of treatment visit Are the trial subjects under 18? yes Number of subjects for this age range: 60 F.1.2 Adults (18-64 years) yes F.1.2.1 Number of subjects for this age range 5 F.1.3 Elderly (>=65 years) no F.1.3.1 Number of subjects for this age range
Exclusion criteria
Exclusion criteria: For new patients: 1. AST and/or ALT >1.5 x ULN at Visit 1. 2. Bilirubin >1.5 x ULN at Visit 1. 3. eGFR 2 ii. Prolongation of prothrombin time (PT) by >1.5 x ULN iii. 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, R
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Main Objective: The trial will assess the safety and tolerability of long-term treatment with nintedanib in pediatric patients with clinically significant fibrosing ILD. The primary objective is to estimate the incidence of treatment emergent adverse events over the whole trial.;Secondary Objective: Not applicable;Primary end point(s): 1) The primary endpoint is the incidence of treatment emergent adverse events over the whole trial.;Timepoint(s) of evaluation of this end point: 1) Whole trial duration | — |
Secondary
| Measure | Time frame |
|---|---|
| Secondary end point(s): None;Timepoint(s) of evaluation of this end point: None | — |
Countries
Argentina, Belgium, Brazil, Canada, Czechia, Czech Republic, Denmark, Finland, France, Germany, Greece, Hungary, Italy, Mexico, Netherlands, Norway, Poland, Portugal, Russian Federation, Spain, Ukraine, United Kingdom, United States
Contacts
Public ContactCT Disclosure & Data Transparency
Boehringer Ingelheim Pharma GmbH&Co KG
Outcome results
None listed