'Insterstitial Lung disease' 'lung fibrosis'
Conditions
Interventions
None listed
Sponsors
Amsterdam UMC
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: To be eligible to participate in this study, subjects must meet all of the following criteria: Arm 1 - IPF/FPF diagnosis (non-stratified) within 1 year prior to screening, based on the guidelines set by ATS/ERS/JRS/ALAT (Raghu et al., 2022)(Zhang & Newton, 2021). A chest HRCT should have been performed within 12 months prior to screening, meeting the minimum requirements for IPF diagnosis by multidisciplinary consultation consensus in the ILD-expertise center based on HRCT or HRCT and lung biopsy if available. If no HRCT is available prior to screening, it can be performed at the screening; - Meeting all of the following criteria during the screening period: 1. FVC >=45% predicted for normal. 2. FEV1/FVC >=0.7. 3. DLco corrected for Hb >=40% predicted of normal. - Able to provide written informed consent as approved by the independent ethics committee; - Able to undergo a CT scan and perform pulmonary function testing; - Age >18 years and =45% predicted for normal. 2. FEV1/FVC >=0.7. 3. DLco corrected for Hb >=40% predicted of normal. - Able to provide written informed consent as approved by the independent ethics committee; - Able to undergo a CT scan and perform pulmonary function testing; - Age >18 years and =45% predicted for normal. 2. FEV1/FVC >=0.7. 3. DLco corrected for Hb >=40% predicted of normal. - Able to provide written informed consent as approved by the independent ethics committee; - Able to undergo a CT scan and perform pulmonary function testing; - Age >18 years and <
Exclusion criteria
Exclusion criteria: A potential subject who meets any of the following criteria will be excluded from participation in this study: Arm 1 - Combined pulmonary fibrosis and emphysema (CPFE) is defined by the coexistence of pulmonary fibrosis and emphysema, as described in the latest paper by Cottin et al. (2022). To meet the criteria for CPFE on high-resolution computed tomography (HRCT), patients must exhibit: emphysema of any subtype, characterized by well-demarcated areas of low attenuation delimited by a very thin wall (
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| - Pulmonary function test. - Inflammatory and Fibrosis Extent assessed by High-resolution Computed Tomography (HRCT) analyzed by using artificial intelligence software. - Biomarkers related to pulmonary fibrosis will be measured in plasma and serum. - Peripheral Blood Mononuclear Cell (PBMC) populations in blood. - Exhaled breath analysis including volatile organic compounds (VOCs) analysis by gas chromatography-mass spectrometry (GC-MS). | — |
Secondary
| Measure | Time frame |
|---|---|
| - Disease-relevant questionnaires. - Genomics, epigenomics, and transcriptome analysis in blood. - Biomarkers related to pulmonary fibrosis will be measured in bronchoalveolar lavage fluid (BALF), if it has been collected at clinical grounds or has been performed in the subgroups. - Lung tissue will be analyzed with histochemical techniques if available from clinical context e.g., diagnostic biopsies or lung explants. - A subgroup of participants will be sampled using a ReCIVA breath analyzer from Owlstone and using a PExA instrument from PExA. - External exposome analyses of thephysical/chemical environment. - Biomarkers related to pulmonary fibrosis will be measured in a 24-hour urine collection. - Metabolome analyses in urine and blood. - Microbiome analyses in stool and nasal swabs. | — |
Countries
Netherlands
Outcome results
None listed