Intervention: Pirfenidone
Trial phase: Phase 2
ATC code: L04AX05
SMILES: Cc1ccc(=O)n(-c2ccccc2)c1
Sex: All
Age groups: Adult | Older Adult
Healthy volunteers: No
Participants at risk: 127
Group description: Participants received pirfenidone 267 mg capsule three times a day from Day 1 to 7 followed by 2 capsules three times a day from Day 8 to 14 then 3 capsules three times a day from Day 15 up to Week 24.
Eligibility criteria:
Inclusion Criteria:

Age >= 18-85 years
Confirmed fibrosing ILD which, following multidisciplinary team review, cannot be classified with either high or moderate confidence as a specific idiopathic interstitial pneumonia or other defined ILD
Progressive disease as considered by the investigator as participants deterioration within the last 6 months, which is defined as a rate of decline in forced vital capacity (FVC) >5% or a significant symptomatic worsening not due to cardiac, pulmonary vascular or other causes
Extent of fibrosis >10% on high-resolution computed tomography
Forced vital capacity >= 45% of predicted value
Diffusing capacity of the lung for carbon monoxide (DLco) >= 30% of predicted value
Forced expiratory volume in 1 second/FVC ratio >= 0.7
Able to do 6-minute walk distance (6MWD) >= 150 meters
For women of childbearing potential: agreement to remain abstinent or use a non-hormonal or hormonal contraceptive method with a failure rate of < 1% per year during the treatment period and for at least 90 days after the last dose of pirfenidone
For men, agreement to remain abstinent or use contraceptive measures, and agreement to refrain from donating sperm

Exclusion Criteria:

Diagnosis with moderate or high confidence of nonspecific interstitial pneumonia and any ILD with an identifiable cause such as connective tissue disease-ILD, chronic hypersensitivity pneumonitis, or others
Diagnosis of idiopathic pulmonary fibrosis independent of the confidence level
History of unstable angina or myocardial infarction during the previous 6 months
Treatment with high dose systemic corticosteroids, or any immunosuppressant other than mycophenolate mofetil/acid (MMF), at any time within the 4 weeks of the screening period. Participants being treated with MMF should be on a stable dose that is expected to remain stable throughout the trial and was started at least 3 months prior to screening
Participants previously treated with pirfenidone or nintedanib
Participants treated with N-acetyl-cysteine for fibrotic lung disease, at any time within the 4 weeks of the screening period
Drug treatment for any type of pulmonary hypertension
Participation in a trial of an investigational medicinal product within the last 4 weeks
Significant other organ co-morbidity including hepatic or renal impairment
Predicted life expectancy < 12 months or on an active transplant waiting list
Use of any tobacco product in the 12 weeks prior to the start of screening, or any unwillingness to abstain from their use through to the Follow-up Visit
Illicit drug or alcohol abuse within 12 months prior to screening
Planned major surgery during the trial
Hypersensitivity to the active substance or to any of the excipients of pirfenidone
History of angioedema
Concomitant use of fluvoxamine
Clinical evidence of any active infection
Any history of hepatic impairment, elevation of transaminase enzymes, or liver function test results as: Total bilirubin above the upper limit of normal (ULN), Aspartate aminotransferase or alanine aminotransferase >1.5 × ULN, and Alkaline phosphatase >2.0 × ULN
Creatinine clearance < 30 milliliter (mL) per minute, calculated using the Cockcroft-Gault formula
Any serious medical condition, clinically significant abnormality on an Electrocardiogram (ECG) at screening, or laboratory test results
An ECG with a heart rate corrected QT interval using Fridericia's formula as >= 500 milliseconds at screening, or a family or personal history of long QT syndrome