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.
Study Type
OBSERVATIONAL
Enrollment
610
The First Affiliated Hospital of Shandong First Medical University
Jinan, Shandong, China
RECRUITINGEstablish the correlation between Age (years) and progression to PPF
Time frame: Up to 12 months
Establish the correlation between Gender (the proportion of female and the proportion of male) and progression to PPF
Time frame: Up to 12 months
Establish the correlation between Body mass index (BMI, kg/m2) and progression to PPF
Time frame: Up to 12 months
Establish the correlation between ILD-Gender-Age-Physiology score (0-3,4-5 and 6-8) and progression to PPF
Time frame: Up to 12 months
Establish the correlation between Artery blood gas and progression to PPF
Artery blood gas: PaCO2 (mmHg) and PaO2 (mmHg)
Time frame: 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)
Time frame: Up to 12 months
Establish the correlation between 6 min walk test distance (meters) and progression to PPF
Time frame: 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)
Time frame: Up to 12 months
Prevalence of PPF
Prevalence of PPF in patients with fibrosing ILD.
Time frame: Up to 36 months
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