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…
Eligibility
- Age range
- 18–80 years
- Sex
- All
- Healthy volunteers
- No
Inclusion Criteria: * 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. Exclusion Criteria: * Site diagnosis of fibrosing ILD \>5 years prior to Visit 1 (Screening and Baseline Visit). * Minimal ILD, defined as reticular opacities and/or ground-glass opacities without architectural distortion (traction bronchiolectasis/bronchiectasis or honeycombing) affecting \< 5%…
Locations (13)
- University of Massachusetts Chan Medical SchoolWorcester, Massachusetts
- University of MichiganAnn Arbor, Michigan
- University of Texas SouthwesternDallas, Texas
- University of VirginiaCharlottesville, Virginia
- University of WashingtonSeattle, Washington
- Royal Prince Alfred HospitalCamperdown, New South Wales