A Multicenter Prospective Study of Risk Factors in Progressive Pulmonary Fibrosis

Study Overview
=================
Brief Summary
-----------------
The goal of this observational study is to learn about risk of progressive pulmonary fibrosis (PPF). The main questions it aims to answer are: Risk factors of PPF Prevalence of PPF Mortality of PPF Patients with interstitial lung disease (ILD) of known or unknown etiology other than IPF who has radiological evidence of pulmonary fibrosis will enroll in this study. All participants will have baseline investigations at the first visit having provided informed consent. At the first visit, baseline characteristics will be collected including demographics, medical history, smoking history, complications and medication use. 50 mL of blood will be obtained. High resolution computed tomography (HRCT), full lung function tests and a 6 min walk test will be performed. Further visits at 6 months and 12 months will include further 50 mL blood sampling. HRCT, full lung function tests and a 6 min walk test will be repeated.

Official Title
-----------------
A Multicenter Prospective Study of Risk Factors in Progressive Pulmonary Fibrosis

Conditions
-----------------
Interstitial Lung Disease

Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: 18-80 years old Agree and sign the informed consent form Interstitial lung disease patients with imaging features of pulmonary fibrosis Exclusion Criteria: Patients with idiopathic pulmonary fibrosis (IPF) Pregnant or lactating women mental illness or cognitive impairment participating in other clinical studies Unable to sign informed consent form

Ages Eligible for Study
-----------------
Minimum Age: 18 Years
Maximum Age: 80 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| PF<br>Patients with interstitial lung disease (ILD) of known or unknown etiology other than IPF who has radiological evidence of pulmonary fibrosis(PF). | |

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Establish the correlation between Age (years) and progression to PPF |  | Up to 12 months | 
| Establish the correlation between Gender (the proportion of female and the proportion of male) and progression to PPF |  | Up to 12 months | 
| Establish the correlation between Body mass index (BMI, kg/m2) and progression to PPF |  | Up to 12 months | 
| Establish the correlation between ILD-Gender-Age-Physiology score (0-3,4-5 and 6-8) and progression to PPF |  | Up to 12 months | 
| Establish the correlation between Artery blood gas and progression to PPF | Artery blood gas: PaCO2 (mmHg) and PaO2 (mmHg) | Up to 12 months | 
| Establish the correlation between Pulmonary function and progression to PPF | Pulmonary function: forced vital capacity (FVC, L) and diffusion capacity for carbon monoxide of the lung (DLCO, mmol/min/kPa) | Up to 12 months | 
| Establish the correlation between 6 min walk test distance (meters) and progression to PPF |  | Up to 12 months | 
| Establish the correlation between Biomarkers and progression to PPF | Biomarkers:CCL18(pg/mL),KL-6(pg/mL),CA125(pg/mL),MMP-7(pg/mL),SP-D(pg/mL) and IL-6(pg/mL) | Up to 12 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Prevalence of PPF | Prevalence of PPF in patients with fibrosing ILD. | Up to 36 months | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
progressive pulmonary fibrosis