P4O2 ILD Extension

Early Identification of Progressive Pulmonary Fibrosis, Precision Medicine for More Oxygen - ILD Extension.

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.

Included participants will complete several study visits to collect clinical data and biological samples. Study visits will be performed at baseline, 3, 6, 12, 24, 36, 48 and 60 months, with a 2-month time window for follow-up visits. These time points are aligned with the standard clinical follow-up visits outlined in the ILD Care Path Protocol of Amsterdam UMC.

Inclusion Criteria:

• 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).

• Meeting all the following criteria during the screening period:

  1. FVC ≥45% predicted.
  2. FEV1/FVC ≥0.7.
  3. DLco corrected for Hb ≥40% predicted.

• Able to provide written informed consent as approved by the independent ethics committee.

• Able to undergo a CT scan and perform PFT.

• Age > 18 years and < 80 years.

• Understanding of the Dutch or English language.

Exclusion Criteria:

• 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.
• Pregnancy or lactating.