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

Inclusion Criteria

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.

Exclusion Criteria

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) 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 patie