build-up of scar tissue in the lungs Idiopathic pulmonary fibrosis (IPF)
Conditions
Interventions
The research is divided into part A and part B. Part A lasts 24 weeks
(approximately 6 months). Part B lasts 48 weeks (about 1 year).
Patients will be assigned to one of the following treatments:
* 5
* 100 mg AP01 twice daily (at least 4 hours apart).
Sponsors
Avalyn Pharma, Inc.
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: 1. Male and female patients, at least 40 years of age at Screening 2. Not eligible for oral pirfenidone and nintedanib due to national formulary; restrictions OR intolerant to or unwilling to start oral pirfenidone and nintedanib, if previously offered; 3. Clinical symptoms consistent with IPF of * 12 months duration (with or without IPF diagnosis) 4. Diagnosis of IPF, defined as the first instance in which a patient was informed of having IPF, no more than 60 months before randomization; Patients that have had an IPF diagnosis * 1 year, the following criteria must be met: - HRCT and/or Surgical Lung Biopsy findings consistent with UIP. If honeycombing is not present on the HRCT, then one or both of the following criteria must be present: * Disease progression since diagnosis by HRCT and/or * An absolute loss of FVC * 5% percent predicted over the past 12 months, Patients that have had IPF diagnosis within the last year, the following criteria must be met: - Diagnosis of Usual Interstitial Pneumonia (UIP) or IPF by HRCT (HRCT must be performed within 12 months prior to Screening) and/or Surgical Lung Biopsy 5. Extent of fibrotic changes (honeycombing, reticular changes) greater than the extent of emphysema on HRCT scan, confirmed by central review; 6. No features supporting an alternative diagnosis on transbronchial biopsy, BAL, or surgical lung biopsy, if performed; 7. 40% * FVC * 90 % predicted at Screening based on Global Lung Initiative12 equations. The first 20 patients randomized must have FVC * 50% predicted. After the first 20 patients have randomized, patients with FVC 40% - 50% predicted will be allowed to be randomized in the study but randomization for these patients will be capped at 20; 8. Change in FVC (measured in liters) between Screening and Day 1 (pre-dose measurement) must be a
Exclusion criteria
Exclusion criteria: 1. Significant clinical worsening of IPF between Screening and Day 1, in the opinion of the investigator; 2. Not a suitable candidate for enrollment or unlikely to comply with the requirements of this study, in the opinion of the investigator; 3. History of acute IPF exacerbation requiring hospitalization in the last3 months; 4. History of clinically significant environmental exposure known to cause pulmonary fibrosis, including but not limited to drugs (such as amiodarone), asbestos, beryllium, radiation, and domestic birds; 5. Known explanation for interstitial lung disease, including but not limited to radiation, drug toxicity, sarcoidosis, hypersensitivity pneumonitis, bronchiolitis obliterans organizing pneumonia, human immunodeficiency virus, viral hepatitis, and cancer; 6. Clinical diagnosis of any connective tissue disease, including but not limited to scleroderma, polymyositis/dermatomyositis, systemic lupus erythematosus, and rheumatoid arthritis; 7. Current diagnosis of asthma or chronic obstructive pulmonary disease; 8. Clinical evidence of active infection, including but not limited to bronchitis, pneumonia, sinusitis, urinary tract infection, or cellulitis; 9. Females with a positive pregnancy test at Screening or are currently breastfeeding 10. Any history of malignancy likely to result in significant disability or likely to require significant medical or surgical intervention within the next 6 months. This does not include minor surgical procedures for localized cancer (e.g., basal cell carcinoma); 11. Any condition other than IPF that, in the opinion of the investigator, is likely to result in the death of the patient within the next 6 months; 12. History of severe hepatic impairment or end-stage liver disease or ALT or AST greater than 5 times the upper limit of normal at Screening; 13. History of end-stage renal disease requiring dialysis 14. Participation in a clinical study with administration of an investigational drug product within the previous 30 days, or five half-lives of the previously administered investigational product.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| * Treatment-emergent AEs * Change from pre-dose to post-dose FEV1 after initial dose * Treatment-emergent deaths * Treatment-emergent changes in clinical laboratory findings * Changes in vital signs | — |
Secondary
| Measure | Time frame |
|---|---|
| * Change from Baseline in FVC % predicted * Change from Baseline in DLCO * Change from Baseline in Patient Reported Outcomes (PRO) * Change from Baseline in cough frequency and intensity * Change from Baseline in extent of fibrosis and lung volumes | — |
Countries
Netherlands
Outcome results
None listed