PRIME-PPF: PRospective phenotypIng and Multi-omic Endotyping of Progressive Pulmonary Fibrosis
This is a prospective, observational cohort study. Participants with non-idiopatic pulmonary fibrosis, interstitial lung disease (ILD) will be followed for 24 months to systematically collect clinical, imaging, and biospecimen data. The primary objective is to optimize progressive pulmonary fibrosis (PPF) classification and establish PPF incidence for key ILD subtypes. Additional exploratory objectives are to 1) Prospectively validate a novel PPF classifier and assess performance durability over time, and 2) Determine whether multi-dimensional PPF prediction outperforms component approaches.
• Age 18-80 years with a diagnosis of non-IPF fibrosing ILD due to CTD-ILD, fHP, or non-IPF IIP based on central review
• Diagnosis of Fibrotic ILD as determined by site investigator.
• Willingness to comply with study procedures and follow-up.
• Provide written informed consent.