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.
The P4O2 ILD-extension is a non-interventional prospective observational cohort study that will include a minimum of 450 participants, divided intro three groups (1) 150 participants diagnosed with idiopathic pulmonary fibrosis (IPF) or familial pulmonary fibrosis (FPF); (2) 150 participants diagnosed with other fibrotic ILDs (fILD), including fibrotic hypersensitivity pneumonitis (fHP), idiopathic non-specific interstitial pneumonia (iNSIP), connective tissue disease (CTD)-ILD, and unclassifiable ILD (uILD); and (3) 150 participants diagnosed with interstitial lung abnormalities (ILA).
Cohort
Prospective
No Interventions
Other interventionNo intervention so not applicable.
Inflammatory and fibrosis extent assessed by HRCT
High-Resolution Computed Tomography (HRCT) is an advanced imaging technique used to obtain detailed images of the lungs and chest. Unlike standard CT scans, HRCT uses thin slices and special algorithms to produce high-resolution images that provide more precise visualization of lung structures, making it particularly useful for diagnosing and evaluating various lung conditions. The Inflammatory and Fibrosis extent will be analyzed by using artificial intelligence software.
Pulmonary function tests (PFTs)- Spirometry volumes
Pulmonary function tests (PFTs) are a group of tests that measure how well your lungs are working. These tests assess lung volume, capacity, rates of flow, and gas exchange. We will look at the following values: 1\. Spirometry Values * Forced Vital Capacity (FVC): The total amount of air exhaled after taking the deepest breath possible. * Forced Expiratory Volume in 1 Second (FEV1): The amount of air forcefully exhaled in one second.
Pulmonary function tests (PFTs)- DLCO measurement
Pulmonary function tests (PFTs) are a group of tests that measure how well your lungs are working. These tests assess lung volume, capacity, rates of flow, and gas exchange. We will look at the Diffusion Capacity Tests (DLCO). DLCO: Measures how well oxygen and CO2 are exchanged between the lungs and the blood.
Pulmonary function tests (PFTs)- Lungvolume measurement
Pulmonary function tests (PFTs) are a group of tests that measure how well your lungs are working. These tests assess lung volume, capacity, rates of flow, and gas exchange. We will look at the Lung Volumes and Capacities * Total Lung Capacity (TLC): The total volume of air in the lungs after taking the deepest breath. * Residual Volume (RV): The amount of air left in the lungs after a full exhalation. * Functional Residual Capacity (FRC): The amount of air remaining in the lungs after normal exhalation. * Inspiratory Capacity (IC): The maximum amount of air that can be inhaled after a normal exhalation.
Disease-relevant questionnaires: Exposure Questionnaire
During multiple visits, participants will be requested to complete the questionnaires below. The questionnaires that are not completed in the study center will be sent to the participants via email to fill out at home or bring to the clinic next visit. The Exposure questionnaire. Participants will be asked about their exposures and exposure risks. They are requested to answer the questions as completely as possible. The participant is asked about their current occupation and occupation history, as well as any present or past exposures to metals, fibers, chemicals, dust, smoke, fog/mist, gases, fumes, animal- or plant-materials, bacterial, viral or fungi contacts. Additionally, we will inquire about what kind of environment they have been living in and the duration of their residency. Other questions will cover hobbies, pesticide contact, and animal contact. The follow-up questionnaire has fewer questions.
Disease-relevant questionnaires: KBILD questionnaire.
During multiple visits, participants will be requested to complete the questionnaires below. The questionnaires that are not completed in the study center will be sent to the participants via email to fill out at home. The King's Brief Interstitial Lung Disease (KBILD) questionnaire is designed to assess the impact of the ILD on the participant's overall well-being and daily life. It can be used to monitor the progression of ILD, evaluate treatment effectiveness, and assess ongoing management. The KBILD questionnaire consists of 15 brief items. For more details, please visit https://www.kbild.com.
Disease-relevant questionnaires: Modified Medical Research Council Dyspnea score
During multiple visits, participants will be requested to complete the questionnaires below. The questionnaires that are not completed in the study center will be sent to the participants via email to fill out at home. The mMRC (Modified Medical Research Council) Dyspnea Scale is used to assess the level of baseline functional disability caused by dyspnea. The mMRC dyspnea scale ranges from grade 0 to 4, with 0 indicating minimal dyspnea. This longitudinal data is valuable for understanding the natural progression of ILDs and evaluating the effectiveness of long-term interventions.
Disease-relevant questionnaires: Visual analog scale (VAS)
During multiple visits, participants will be requested to complete the questionnaires below. The questionnaires that are not completed in the study center will be sent to the participants via email to fill out at home. The Visual analog scale (VAS) is a linear scoring method with a scale ranging from 0 to 10. A score of 0 represents no coughing symptoms, while a score of 10 represents the most severe coughing. The participant is asked to mark the severity of the cough on the scale based on self-perception.
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Amsterdam UMC, location VUmc
Lead sponsor
Academisch Medisch Centrum - Universiteit van Amsterdam (AMC-UvA)
Sponsor institution
AbbVie
Collaborator
Boehringer Ingelheim
Collaborator
