A Study of Pirfenidone in Patients With Unclassifiable Progressive Fibrosing Interstitial Lung Disease

This is an interventional treatment trial for Lung Diseases, Interstitial Read More

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