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

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 Overview

• Status: Recruiting
• Conditions: Progressive Pulmonary Fibrosis

• Study Type: Observational
• Enrollment (Estimated): 600

Contacts and Locations

• Study Contact: Name: Zuojun Xu Zuojun Xu; Phone Number: +86 13671345136; Email: xuzi@hotmail.com

Study Locations

• China
  • Beijing, China
    • Recruiting
    • Peking Union Medical College Hospital
    • Contact: Zuojun Xu; Phone Number: +86 13671345136; Email: xuzi@hotmail.com

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child; Adult; Older Adult
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

Patients diagnosed with PPF in China

Description

Inclusion Criteria:

1. Diagnosis of PPF within the past year, according to the 2022 ATS/ERS/JRS/ALAT guidelines;
2. Patients who are willing to participate in the study and have signed an informed consent form.

Exclusion Criteria:

1. Patients diagnosed with IPF.
2. Patients without baseline chest HRCT.
3. Patients who refuse to sign the informed consent form.
4. 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.)

Study Plan

How is the study designed?

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort
Chinese PPF Prospective Registry Cohort

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Absolute change in FVC (mL)	Absolute change in FVC (mL) at 1-year and 2-year follow-up.	at 1-year and 2-year follow-up

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
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.	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: Worsening respiratory symptoms;; Physiological evidence of disease progression (FVC decline >5% or DLCO; 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).	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.	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.	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.	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.	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.	during the 2-year follow-up
Time to first acute exacerbation	Time to first acute exacerbation during the 2-year follow-up.	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.	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	at 2-year follow-up

Collaborators and Investigators

• Sponsor: Zuojun Xu
• Collaborators: Boehringer Ingelheim

Study record dates

Study Major Dates

• Study Start (Actual): September 23, 2025
• Primary Completion (Estimated): July 31, 2029
• Study Completion (Estimated): October 31, 2029

Study Registration Dates

• First Submitted: June 25, 2026
• First Submitted That Met QC Criteria: July 1, 2026
• First Posted (Actual): July 6, 2026

Study Record Updates

• Last Update Posted (Actual): July 6, 2026
• Last Update Submitted That Met QC Criteria: July 1, 2026
• Last Verified: July 1, 2026

More Information

Terms related to this study

• Keywords: Registry Study; Progressive Pulmonary Fibrosis
• Other Study ID Numbers: PROFINA

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: UNDECIDED

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No