PRIME-PPF: PRospective phenotypIng and Multi-omic Endotyping of Progressive Pulmonary Fibrosis
University of Massachusetts, Worcester
Summary
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.
Description
This is a prospective, cohort study with no study investigational therapy prescribed. Patients with non-idiopathic pulmonary fibrosis (non-IPF) fibrosing ILD, including connective tissue disease associated ILD (CTD-ILD), fibrotic hypersensitivity pneumonitis (fHP) and non-IPF idiopathic interstitial pneumonia (IIP) will be recruited. Consented participants with non-IPF ILD will be carefully phenotyped with extensive clinical, physiological, and imaging data. After confirmation of eligibility, participants will undergo protocolized follow-up over 24 months. Clinical data and blood biospecimens…