Pulmonary Fibrosis, Sarcoidosis
Conditions
Brief summary
FIBRALUNG is a prospective cohort study with biobank of samples from patients with pulmonary fibrosis, aiming to explore the molecular determinants of different clinical outcomes, acute exacerbations and mortality. We expect to gain deeper insight into fibroproliferative common pathways, particularly between idiopathic pulmonary fibrosis and fibrotic hypersensitivity pneumonitis, paving the way for new biomarkers that reflect the progressive phenotype, that eventually will support new targeted therapies. Other idiopathic interstitial pneumonias, connective tissue disease-related interstitial lung diseases and sarcoidosis patients will be also recruited and their biological samples stored for further analyses.
Interventions
OTHERComprehensive clinical assessment and biological samples collection
To establish the first Portuguese registry and biobank of PF-ILDs, comprising both extensive patient-level data, and systematic biological sampling (DNA, RNA, plasma, serum, bronchoalveolar lavage, lung tissue) at baseline and repeated biological sampling of blood and pharyngeal swabs performed at 6, 12 and 18 months, or whenever progression criteria are met or an acute exacerbation occurs. Participants will have regular visits at maximum intervals of 6 months, when their clinical condition and lung function tests are reassessed. A high resolution computed tomography (HRCT) scan of the lung will be performed every 12 months. Progressive fibrosis will be diagnosed based on meeting at least two of the following three criteria, occurring within the last year: (i) worsening of symptoms; (ii) absolute decline in FVC ≥5% predicted or absolute decline in DLCO (corrected for Hb) ≥10% predicted; (iii) increased extent of fibrotic changes on HRCT.
Sponsors
Centro Hospitalar De São João, E.P.E.
Instituto de Investigação e Inovação em Saúde (i3S)
Fundação para a Ciência e a Tecnologia
Universidade do Porto
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to 80 Years
Healthy volunteers
Yes
Inclusion criteria
* Patients aged between 18-80 years * People undergoing blood collection, lung biopsy and/or BAL as part of their diagnostic workup * Willingness to undergo the follow-up protocol evaluations * Treatment-naïve for disease-modifying drugs * An HRCT scan performed within the last 12 months showing ≥10% fibrosis extent of the lungs
Exclusion criteria
* People who cannot give informed consent * Pregnancy
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Discover biomarkers in progressive pulmonary fibrosis | 36 months | Characterization of blood and tissue transcriptional signatures of progression and acute exacerbations, and validate findings at the protein expression level, which could be easily converted for clinical use as biomarkers. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Change in microbiome profile in progressive pulmonary fibrosis | 24 months | To assess the impact of microbiome features in clinical progression and higher risk of acute exacerbation |
Other
| Measure | Time frame |
|---|---|
| Proportion of patients varying DLCO ⩾10% predicted within 1 year of follow-up | 36 months |
| Variation in Computed Tomography Lung Densitometry | 36 months |
| Survival | 36 months |
| Time to progression or exacerbation | 36 months |
| Proportion of patients varying FVC ⩾5% predicted within 1 year of follow-up | 36 months |
Countries
Portugal
Contacts
Primary ContactHelder Novais Bastos, MD, PhD
Backup ContactJanete Santos, PhD
Outcome results
None listed