The goal of this observational study is to identify early biomarkers that can predict the development of progressive pulmonary fibrosis (PPF) in participants with interstitial lung diseases (ILDs). The participant population includes adults diagnosed with idiopathic pulmonary fibrosis (IPF), familial pulmonary fibrosis (FPF), other fibrotic ILDs, and interstitial lung abnormalities (ILA).
The main questions it aims to answer are:
* What biomarkers and risk factors are linked to fibrosis progression or can predict rapid worsening and sudden flare-ups in IPF and FPF patients? * What biomarkers and risk factors can predict the development of a PPF phenotype in different types of ILD? * What biomarkers and risk factors can help identify ILA patients who may develop significant ILD? * What biomarkers and risk factors can predict how well ILD patients will respond to treatment?
Researchers will compare the outcomes between participants diagnosed with IPF/FPF, other fibrotic ILDs, and ILA to see if early detection biomarkers differ among these groups.
Participants will:
* Undergo blood sampling. * Perform lung function tests. * Have CT scans. * Perform breath analysis * Participate in exposome and microbiome analyses. * Complete questionnaires. * A subgroup of participants will be offered bronchoscopy.
Diagnosis of (1) idiopathic pulmonary fibrosis (IPF), familial pulmonary fibrosis (FPF), (2) other fibrotic ILDs (fILD), including fibrotic hypersensitivity pneumonitis (fHP), idiopathic non-specific interstitial pneumonia (iNSIP), connective tissue disease (CTD)-ILD, and unclassifiable ILD (uILD); or (3) interstitial lung abnormalities (ILA).
FVC ≥45% predicted.
FEV1/FVC ≥0.7.
DLco corrected for Hb ≥40% predicted.
Combined pulmonary fibrosis and emphysema (CPFE) diagnosis
Chronic obstructive lung disease (COPD) with an FEV1/FVC <70%.
Uncontrolled severe asthma.
Active malignancy, except for squamous cell carcinoma of the skin, low-risk breast cancer, and low-risk prostate cancer.
Amsterdam UMC, location VUmc
Lead sponsor
Academisch Medisch Centrum - Universiteit van Amsterdam (AMC-UvA)
Sponsor institution
AbbVie
Collaborator
Boehringer Ingelheim
Collaborator