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.
Diagnosis of PPF within the past year, according to the 2022 ATS/ERS/JRS/ALAT guidelines;
Patients who are willing to participate in the study and have signed an informed consent form.
Patients diagnosed with IPF.
Patients without baseline chest HRCT.
Patients who refuse to sign the informed consent form.
Patients participating in other clinical trials for medications. (Patients who have completed other trials or are in an unblinded phase after a washout period of 2 weeks are eligible for enrollment.)
Patients diagnosed with PPF in China
Cohort
Prospective
Not listed
Absolute change in FVC (mL)
Absolute change in FVC (mL) 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.
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).
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.
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.
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Zuojun Xu
Lead sponsor
Peking Union Medical College Hospital
Sponsor institution
Boehringer Ingelheim
Collaborator
