This is a nationwide multicenter prospective non-interventional registry study enrolling 600 Chinese patients diagnosed with PPF over a 3-year period (9-month enrollment, 2-year follow-up, and 3 months for data analysis and publication). Eligible participants must meet the PPF criteria defined by the 2022 ATS/ERS/JRS/ALAT guidelines, while patients with IPF, no baseline chest HRCT, or refusal to sign informed consent will be excluded. The primary endpoint is the absolute change in FVC (mL) at 1- and 2-year follow-ups. The core objective is to investigate real-world disease progression in Chinese PPF patients, with secondary objectives to analyze their clinical characteristics, current treatment status, and unmet clinical needs.
Study Type
OBSERVATIONAL
Enrollment
600
Peking Union Medical College Hospital
Beijing, China
RECRUITINGAbsolute change in FVC (mL)
Absolute change in FVC (mL) at 1-year and 2-year follow-up.
Time frame: at 1-year and 2-year follow-up
CTD-ILD, HP, iNSIP, exposure-related ILD, sarcoidosis, unclassified ILD, and other ILDs
The percentage of patients with CTD-ILD, HP, iNSIP, exposure-related ILD, sarcoidosis, unclassified ILD, and other ILDs at baseline and following 2 years of follow-up.
Time frame: at baseline and after 2 years of follow-up
The percentage of patients who meet at least two of the following three inclusion criteria for PPF
The percentage of patients who meet at least two of the following three inclusion criteria for PPF at baseline, 1-year, and 2-year follow-up: 1. Worsening respiratory symptoms; 2. Physiological evidence of disease progression (FVC decline \>5% or DLCO 3. Imaging evidence of disease progression (e.g., increased or worsened traction bronchiectasis, new ground-glass opacities with traction bronchiectasis, new reticular patterns, or new honeycombing).
Time frame: at baseline, 1-year, and 2-year follow-up
The percentage of patients receiving treatment with corticosteroids, immunosuppressive agents, and antifibrotic drugs or PDE4B inhibitors
The percentage of patients receiving treatment with corticosteroids, immunosuppressive agents (including cyclophosphamide, mycophenolate mofetil, azathioprine, and other commonly used immunosuppressants for ILDs), and antifibrotic drugs (nintedanib, pirfenidone) or PDE4B inhibitors (e.g., nerandomilast) during the 2-year follow-up period.
Time frame: during the 2-year follow-up period
Change in absolute DLCO of pulmonary function expressed as percent predicted
Change in absolute DLCO of pulmonary function expressed as percent predicted after 1 year and 2 years of follow-up.
Time frame: after 1 year and 2 years of follow-up
Changes in the absolute score of the dyspnea severity questionnaire
Changes in the absolute score of the dyspnea severity questionnaire at 1-year and 2-year follow-up.
Time frame: at 1-year and 2-year follow-up
Changes in the absolute score of the Cough Severity Numerical Rating Scale (CNSRS)
Changes in the absolute score of the Cough Severity Numerical Rating Scale (CNSRS) at 1-year and 2-year follow-up.
Time frame: at 1-year and 2-year follow-up
Frequency of acute exacerbations of ILD
Frequency of acute exacerbations of ILD during the 2-year follow-up.
Time frame: during the 2-year follow-up
Time to first acute exacerbation
Time to first acute exacerbation during the 2-year follow-up.
Time frame: during the 2-year follow-up
The percentage of patients with acute exacerbations, lung transplantation, or death
The percentage of patients with acute exacerbations, lung transplantation, or death at 2-year follow-up.
Time frame: at 2-year follow-up
The percentage of patients with ILD progression (FVC decline >10% of predicted value)
The percentage of patients with ILD progression (FVC decline \>10% of predicted value) at 2-year follow-up
Time frame: at 2-year follow-up
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