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A study to evaluate the dose-exposure, safety, and exploratory efficacy of nerandomilast in children and adolescents from 2 years to less than 18 years of age with fibrosing interstitial lung disease (Part A: double-blind, placebo controlled in children from 6 to less than 18 years of age and open label active treatment in children from 2 to less than 6 years of age), followed by an open-label phase with active treatment (Part B)

A study to find out how nerandomilast is tolerated, handled by the body, and if it helps children and adolescents with interstitial lung disease

Mori Hiroko

Boehringer Ingelheim

2-1-1, Osaki, Shinagawa-ku, Tokyo

medchiken.jp@boehringer-ingelheim.com

Furuichi Takumi

Boehringer Ingelheim

2-1-1, Osaki, Shinagawa-ku, Tokyo

+81-120-189-779

medchiken.jp@boehringer-ingelheim.com

Pending

April. 17, 2026

Interventional

randomized controlled trial

double blind

placebo control

parallel assignment

treatment purpose

- Children and adolescents 2 to <18 years old at Visit 2.
- Participants with evidence of fibrosing ILD on high-resolution computed tomography (HRCT) within 12 months of Visit 1 as assessed by the investigator and confirmed by central review.
- For children >=6 years: Participants with forced vital capacity (FVC) % predicted >=25% at Visit 2.
- Participants with clinically significant fibrosing ILD 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).

Further inclusion criteria apply.

- Previous treatment with nerandomilast.
- Participants treated with other oral/systemic PDE4 and non-selective PDE inhibitors within 30 days before Visit 1.
- Participants treated with pirfenidone in the 8 weeks prior to Visit 1.
- Unstable pulmonary arterial hypertension (PAH).
- Active vasculitis, unstable or uncontrolled within 8 weeks prior to Visit 1 or during the screening period.
- Any suicidal behaviour (i.e. actual attempt, interrupted attempt, aborted attempt, or preparatory acts or behaviour) in the past (lifetime).
- Any suicidal ideation of type 4 or 5 on the columbia suicidal severity rating scale (C-SSRS) in the past 3 months at Visit 1 or at Visit 2 (i.e. active suicidal thought with method and intent but without specific plan; or active suicidal thought with method, intent, and plan).
- Participants with clinically significant depression symptoms defined as the short version of mood and feeling questionnaire (SMFQ) score >=8.

Further exclusion criteria apply.

Both

Fibrosing Interstitial Lung Disease

Drug: Nerandomilast
Drug: Placebo

- Area under the concentration curve (AUC) T,SS based on sampling at steady state using rich sampling in participants from 6 years to less than 18 years and sparse sampling in participants younger than 6 years [Time Frame: At week 2 in Part A and Week 28 in Part B]
- Occurrence of a treatment-emergent adverse event [Time Frame: up to Week 26]

- Absolute change from baseline in oxygen saturation (SpO2) [%] on room air at rest [Time Frame: at Week 26 and Week 52]
- Absolute change from baseline in height [cm] [Time Frame: at Week 26 and Week 52]
- Absolute change from baseline in pediatric quality of life inventory ( PedsQL TM) [Time Frame: at Week 26 and Week 52]
- Health-related quality of life will be assessed in children using the PedsQL TM questionnaire. For younger children who are unable to perform self-assessment, a parent proxy may be required. The score ranges from 0-100, a higher score indicates a better health related quality of life.
- Occurrence of a treatment-emergent adverse event (Yes/No) over the whole trial [Time Frame: up to 5 years]
- Time to first respiratory-related hospitalisation [days] over the whole trial [Time Frame: up to 5 years]
- Time to first acute interstitial lung disease (ILD) exacerbation or death [days] over the whole trial [Time Frame: up to 5 years]
- Time to death [days] over the whole trial [Time Frame: up to 5 years]
- Participant acceptability based on number/size of tablets [Time Frame: at Week 2 and Week 26]
- Acceptability is defined as the overall ability and willingness of the participant to use the medicinal product as intended.
- Assessment of acceptability will be performed by the participant using the acceptability questionnaire.
- Participant acceptability based on the use of the dispenser [Time Frame: at Week 2 and Week 26]
- Absolute change from baseline in FVC [% predicted] (applicable to participants >=6 years) [Time Frame: at Week 26 and Week 52]
- Absolute change from baseline in 6-min walk distance [m] (applicable to participants >=6 years) [Time Frame: at Week 26 and Week 52]

+81-3-5494-7297

Yes

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