Prospective Multi-Center Registry Observing Disease Progression and Treatments in Chinese Patients with Progressive Pulmonary Fibrosis

What this Trial Is About

Researchers are conducting a nationwide, multicenter, prospective observational study involving 600 Chinese patients diagnosed with progressive pulmonary fibrosis PPF. The study aims to observe real-world disease progression, clinical characteristics, current treatments, and unmet clinical needs in PPF patients following the 2022 ATSERSJRSALAT guidelines. Patients with idiopathic pulmonary fibrosis IPF, no baseline chest HRCT, or those refusing consent are excluded from participation. This study is planned over a 3-year period, including 9 months of enrollment, 2 years of follow-up, and 3 months for data analysis and publication. During the study, participants are observed without any experimental treatments as this is a non-interventional registry. The main measure is the absolute change in forced vital capacity FVC at 1- and 2-year follow-ups. Secondary analyses include tracking lung function changes, symptom severity scores, treatment use of corticosteroids, immunosuppressives, antifibrotic drugs or PDE4B inhibitors, and the frequency and timing of acute exacerbations or other major clinical events. Participants will undergo regular evaluations over 2 years, including lung function tests, chest CT scans, questionnaires on dyspnea and cough severity, and monitoring of acute exacerbations or lung transplantation events. Data collected will help understand disease progression and treatment patterns in the Chinese PPF population. The total study duration spans from enrollment through 2 years of follow-up and final data analysis.

A Prospective, Multi-Center Registry Study of Progressive Pulmonary Fibrosis (PPF) in China (PROFINA)