A Study Evaluating the Safety and Efficacy of Inhaled AP01 in Participants with Progressive Pulmonary Fibrosis

Eligibility Criteria

• * Participant meets criteria for PPF, as follows:
• * In subjects with interstitial lung disease (ILD) of known or unknown etiology other than idiopathic pulmonary fibrosis (IPF) who have radiological evidence of pulmonary fibrosis, PPF is defined as:
• 1. Relative decline in FVC ≥10% predicted within the previous 24 months compared to Screening Visit 1
• 2. Relative decline in FVC ≥5 to \<10% predicted within the previous 24 months compared to Screening Visit 1 with at least 1 of the 2 following criteria:
• * Worsening respiratory symptoms (Note: Changes attributable to comorbidities e.g., infection, heart failure must be excluded) OR
• * Radiological (HRCT) evidence of disease progression per a local or central radiologist, for example:
• * Increased extent or severity of traction bronchiectasis and bronchiolectasis
• * New ground-glass opacity with traction bronchiectasis
• * New fine reticulation
• * Increased extent or increased coarseness of reticular abnormality
• * New or increased honeycombing
• * Increased lobar volume loss
• 3. Worsening of respiratory symptoms (Note: Changes attributable to comorbidities e.g., infection, heart failure must be excluded) AND radiological (HRCT) evidence of disease progression per a local or central radiologist
• * Meeting all of the following criteria during the Screening Period:
• * Current treatment with oral pirfenidone or treatment with oral pirfenidone within 3 months prior to Screening.
• * Elevated liver enzymes and liver injury at Screening defined as:
• 1. Alanine aminotransferase (ALT) or aspartate aminotransferase (AST) ˃ 3 times the upper limit of normal (ULN)
• 2. Bilirubin \>2.0 x ULN
• * Renal disease with a creatinine clearance \< 30 mL/min, calculated according to the Chronic Kidney Disease Epidemiology Collaboration formula. Retesting is allowed once.
• * Diagnosis of idiopathic pulmonary fibrosis (IPF) based on the ATS diagnostic algorithm for IPF. UIP that is not idiopathic, for example related to rheumatoid arthritis (RA), familial interstitial lung disease (ILD), or other is not exclusionary.
• * Greater extent of emphysema than of fibrotic ILD on HRCT. Note: CT results must be confirmed through the central over read process.
• * Significant clinical worsening of PPF between Screening
• * Participants who cannot meet protocol-specified Baseline stability criteria. FVC Baseline stability is defined as the FVC assessments at Visit 3 being within ±12% of the mean of the FVC assessments obtained at the 2 preceding visits. At Visit 3, if the pre-dose FVC is outside of ±12% range, the participant will not be randomized and will be considered a screen failure.