Familial Pulmonary Fibrosis, Interstitial Lung Abnormalities, Interstitial Lung Diseases
Conditions
Brief summary
This study is open to people aged 40 years or older who have at least 1 family member with pulmonary fibrosis. Pulmonary fibrosis is a condition where lung tissue becomes scarred, making it harder to breathe. People can join if a lung scan shows early changes in the lung, called interstitial lung abnormalities, which may lead to lung scarring. People with family members who have pulmonary fibrosis are more likely to develop it themselves. That is why it is important to check early for lung changes and find ways to prevent the condition from getting worse. The purpose of this study is to find out whether a medicine called nerandomilast can help slow down changes in the lung in people with a family history of pulmonary fibrosis. Participants are put into one of 2 groups randomly, which means the group is chosen by chance. One group takes nerandomilast tablets, and the other group takes placebo tablets. Placebo tablets look like nerandomilast tablets but do not contain any medicine. Participants take a tablet twice a day for about 2 to 3 years. There is a 3 out of 5 chance that participants will receive nerandomilast instead of the placebo. Participants are in the study for about 2 to 3 years. Participants visit the study site multiple times: more frequently during the first 2 years (about every 3 months), and then every 6 months thereafter. In the 3rd year, participants also have phone calls with the site staff every 3 months. Doctors regularly test lung function and take chest scans to see if the treatment works. The results are compared between the 2 groups to see if nerandomilast helps. The doctors also check participants' health and take note of any unwanted effects.
Interventions
DRUGNerandomilast
Nerandomilast
DRUGPlacebo
Placebo
Sponsors
Boehringer Ingelheim
Study design
Allocation
RANDOMIZED
Intervention model
PARALLEL
Primary purpose
TREATMENT
Masking
QUADRUPLE (Subject, Caregiver, Investigator, Outcomes Assessor)
Eligibility
Sex/Gender
ALL
Age
40 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Individuals ≥40 years of age at the time of first signed informed consent at Visit 1a * Participants must have at least 1 first-degree relative (biological parent, sibling, or child) with confirmed pulmonary fibrosis (idiopathic pulmonary fibrosis \[IPF\], idiopathic nonspecific interstitial pneumonia \[NSIP\], and/or pulmonary fibrosis due to known genetic cause \[e.g. short telomere syndrome, mucin 5B (MUC5B) mutation, surfactant protein mutations\]) * High resolution computed tomography (HRCT) scan with evidence of interstitial lung abnormalities involving at least 5% of a single lung zone or interstitial lung disease (ILD), based on central evaluation * Forced vital capacity (FVC) ≥80% of predicted normal at Visit 1b * Diffusing capacity of the lungs for carbon monoxide (DLCO) corrected for hemoglobin ≥70% of predicted normal at Visit 1b Further inclusion criteria apply.
Exclusion criteria
* Prior known pulmonary fibrosis that, in the opinion of the Investigator, requires treatment with approved therapies * Prebronchodilator forced expiratory volume in 1 second (FEV1)/FVC \<0.7 at Visit 1b * HRCT findings consistent with probable or definite usual interstitial pneumonia (UIP) pattern * Any medical condition that is known to predispose to the development of pulmonary fibrosis (e.g. known connective tissue disease) * Prior or current use of nerandomilast, nintedanib, or pirfenidone Further
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Time to physiologic or radiologic worsening of ILA/ILD over the whole trial | up to 164 weeks | Defined as relative decline in forced vital capacity (FVC) % predicted of \>10% from baseline; or absolute decline in diffusing capacity of the lungs for carbon monoxide (DLCO) % predicted \>10% from baseline; or absolute increase in weighted reticulovascular score (wRVS) \>2% and total disease extent (TDE) \>2.5% on chest high resolution CT scan (HRCT), as measured by e-Lung Quantitative HRCT scoring, from baseline |
Secondary
| Measure | Time frame |
|---|---|
| Absolute change from baseline in wRVS on e-Lung Quantitative HRCT scoring at weeks 26, 52, and 104 | at baseline, at weeks 26, 52 and 104 |
| Absolute change from baseline in TDE on e-Lung Quantitative HRCT scoring at weeks 26, 52, and 104 | at baseline, at weeks 26, 52 and 104 |
| Absolute change from baseline in FVC (% predicted) at weeks 26, 52, and 104 | at baseline, at weeks 26, 52 and 104 |
| Absolute change from baseline in DLCO (% predicted) at weeks 26, 52, and 104 | at baseline, at weeks 26, 52 and 104 |
| Time to relative decline from baseline in FVC (% predicted) of >10% over 52 weeks and over the whole trial | up to 164 weeks |
| Time to absolute decline from baseline in FVC (% predicted) of >5% over 52 weeks and over the whole trial | up to 164 weeks |
| Time to absolute decline from baseline in DLCO (% predicted) of >10% over 52 weeks and over the whole trial | up to 164 weeks |
| Time to absolute increase in wRVS >2% and TDE >2.5% on chest HRCT, as measured by e-lung quantitative HRCT scoring over 52 weeks and over the whole trial | up to 164 weeks |
| Time to physiologic or radiologic worsening of ILA/ILD over 52 weeks | up to 52 weeks |
Countries
Argentina, Australia, Belgium, Canada, France, Germany, Italy, Japan, Netherlands, South Korea, Spain, United Kingdom, United States
Contacts
CONTACTBoehringer Ingelheim
Outcome results
None listed