Prospective Study of Progressive Pulmonary Fibrosis in Non-IPF Fibrosing Interstitial Lung Disease Observing Clinical, Imaging, and Biomarker Data Over 24 Months

What this Trial Is About

Researchers are studying people with non-idiopathic pulmonary fibrosis non-IPF fibrosing interstitial lung disease ILD, including connective tissue disease-associated ILD, fibrotic hypersensitivity pneumonitis, and non-IPF idiopathic interstitial pneumonia. This observational study aims to improve the classification of progressive pulmonary fibrosis PPF and determine how often PPF occurs in key ILD types. The study also explores a new PPF classifier and compares it with multi-dimensional prediction methods over time. Participants will be carefully assessed with detailed clinical, physiological, and imaging data. They will be followed for 24 months without any study-prescribed treatment. Blood samples and clinical data will be collected at scheduled times. The main outcome measured is 12-month transplant-free survival TFS after an initial 12-month observation, focusing on lung function decline and other PPF criteria. Researchers will also evaluate a novel proteomic biomarker and compare its accuracy to other prediction approaches. During the study, participants will have regular follow-up visits for clinical assessments, imaging, and blood sample collection. The researchers will monitor lung function and disease progression using tests like pulmonary function tests and high-resolution CT scans. The study will track survival without lung transplant or death over the second year. The total duration of participation is 24 months to gather comprehensive data on disease progression and outcomes.

PRospective phenotypIng and Multi-omic Endotyping of Progressive Pulmonary Fibrosis