Access to Pirfenidone Solution for Inhalation (AP01) for Treatment of Progressive, Fibrosing Interstitial Lung Diseases, including Idiopathic Pulmonary Fibrosis

Prior treatment

• 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 (IP) (AP01 naïve patients only)
• Medical Exclusions 5. Females with a positive pregnancy test at Screening (AP01 naïve patients) or Baseline (Rollover patients from previous study) or are currently breastfeeding

Other illness

• Disease-Related Exclusions 1. Significant clinical worsening of ILD/IPF for AP01 naïve patients between Screening and Day 1, in the opinion of the Investigator
• Hypersensitivity to the active substance or to any of the excipients of pirfenidone
• History of acute IPF exacerbation requiring hospitalization in the last 30 days
• Clinical evidence of active infection, including but not limited to bronchitis, pneumonia, sinusitis, urinary tract infection, or cellulitis
• 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)
• Any condition other than ILD/IPF that, in the opinion of the Investigator, is likely to result in the death of the patient within the next 6 months
• History of end-stage renal disease requiring dialysis

Lab values

• History of severe hepatic impairment, end-stage liver disease, or AST or ALT greater than 5 times the upper limit of normal at Screening (AP01 naïve patients)

Other

• Not a suitable candidate for enrollment or unlikely to comply with the requirements of this study, in the opinion of the Investigator

• Prior participant in an Avalyn AP01 study, excluding normal healthy volunteers OR
• Patients with no other treatment options with ILDs, including but not limited to IPF, fibrosing phenotype ILD, pulmonary involvement of scleroderma, rheumatoid lung and silicosis. Prior Sponsor approval of non-Avalyn study rollover patients is required.
• Diagnosed chronic progressive fibrotic ILD, including IPF, without treatment alternatives such as: a. Not eligible for oral pirfenidone and nintedanib due to national formulary restrictions or lack of applicable regulatory approval b. Intolerant to oral pirfenidone and nintedanib, if previously offered c. Not eligible for an ongoing clinical study of AP01 other than this study
• Age greater than 18 years at Screening
• Able to understand and sign, prior to study entry, a written informed consent form (ICF) consistent with International Council on Harmonisation Guideline for Good Clinical Practice (ICH-GCP) and local laws
• Able to understand the importance of adherence to study treatment and the study protocol and willing to follow all study requirements, including the concomitant medication restrictions, throughout the study
• Females of childbearing potential (FOCBP) must use an effective contraceptive method during the clinical study and 30 days after the last dose of AP01 as described in Section 7.3.6, Pregnancy Testing.