Kingston

This research focuses on participants who previously took part in Avalyn Pharma-sponsored studies involving inhaled antifibrotic agents, such as AP01, for progressive pulmonary fibrosis or idiopathic pulmonary fibrosis. The study aims to evaluate the long-term safety and tolerability of these inhaled antifibrotic medications, continuing from where the prior study ended. It is an open-label extension study allowing participants to continue treatment with AP01. All participants will receive 100 mg of inhaled pirfenidone solution AP01 twice daily delivered through the eFlow Nebulizer System. The study involves a screening and baseline visit, followed by an open-label treatment period where participants continue receiving AP01. After completing treatment, participants will have a follow-up phone call approximately two weeks later to conclude their involvement. During the study, participants will use a paper dosing diary to track adherence, and any unused medication will be returned for monitoring. Researchers will assess safety and tolerability over an average of six years, along with lung function changes, disease stabilization, and quality of life measures. The total duration extends up to the studys end date in 2031, with ongoing safety evaluations.