Fibrosing ILD Biomarkers That Rule Acceleration (FIBRALUNG)

Host-microbiome Interactions in the Quest for Fibrosing ILD Biomarkers That Rule Acceleration

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.

Study Overview

• Status: Recruiting
• Conditions: Pulmonary Fibrosis; Sarcoidosis
• Intervention / Treatment: Other: Comprehensive clinical assessment and biological samples collection

• Study Type: Observational
• Enrollment (Estimated): 150

Contacts and Locations

• Study Contact: Name: Helder Novais Bastos, MD, PhD; Phone Number: (+351) 220408800; Email: hnovaisbastos@med.up.pt
• Study Contact Backup: Name: Janete Santos, PhD; Phone Number: (+351) 225 513 600; Email: investigaclinica@med.up.pt

Study Locations

• Portugal
  • Porto, Portugal
    • Recruiting
    • Centro Hospitalar Universitário São João
    • Contact: Helder Novais Bastos; Email: hnovaisbastos@med.up.pt
    • Sub-Investigator: Rita Santos

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 80 years (Adult, Older Adult)
• Accepts Healthy Volunteers: Yes

• Sampling Method: Non-Probability Sample

Study Population

Participants will be recruited from the Interstitial Lung Diseases unit of Centro Hospitalar Universitário de São João (Porto, Portugal).

Description

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

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

Number of groups / cohorts

3

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
IPF; Patients with Idiophatic Pulmonary Fibrosis (IPF), serving as a prototype of a progressive fibroproliferative disorder.	Other: Comprehensive 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.
Progressive Pulmonary Fibrosis (non-IPF); Patients with non-IPF interstitial lung diseases, presenting a progressive fibrosing phenotype, or acute exacerbations.	Other: Comprehensive 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.
Non-Progressive Pulmonary Fibrosis (non-IPF); Patients with fibrotic non-IPF interstitial lung diseases that are stable during a minimum follow-up of 24 months.	Other: Comprehensive 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.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Discover biomarkers in progressive pulmonary fibrosis	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.	36 months

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Change in microbiome profile in progressive pulmonary fibrosis	To assess the impact of microbiome features in clinical progression and higher risk of acute exacerbation	24 months

Other Outcome Measures

Outcome Measure	Time Frame
Variation in Computed Tomography Lung Densitometry	36 months
Proportion of patients varying FVC ⩾5% predicted within 1 year of follow-up	36 months
Proportion of patients varying DLCO ⩾10% predicted within 1 year of follow-up	36 months
Time to progression or exacerbation	36 months
Survival	36 months

Collaborators and Investigators

• Sponsor: Universidade do Porto
• Collaborators: Fundação para a Ciência e a Tecnologia; Instituto de Investigação e Inovação em Saúde (i3S); Centro Hospitalar Universitário de São João, E.P.E.; Faculty of Medicine (FMUP)
• Investigators: Principal Investigator: Helder Novais Bastos, MD, PhD, Faculty of Medicine (FMUP)

Publications and helpful links

Helpful Links

• Project presentation website

Study record dates

Study Major Dates

• Study Start (Actual): March 1, 2021
• Primary Completion (Estimated): February 29, 2024
• Study Completion (Estimated): March 31, 2026

Study Registration Dates

• First Submitted: November 22, 2022
• First Submitted That Met QC Criteria: November 22, 2022
• First Posted (Actual): December 2, 2022

Study Record Updates

• Last Update Posted (Actual): October 27, 2023
• Last Update Submitted That Met QC Criteria: October 24, 2023
• Last Verified: November 1, 2022

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Pathologic Processes; Respiratory Tract Diseases; Immune System Diseases; Lymphoproliferative Disorders; Lymphatic Diseases; Lung Diseases; Hypersensitivity; Fibrosis; Lung Diseases, Interstitial; Hypersensitivity, Delayed; Pulmonary Fibrosis; Sarcoidosis
• Other Study ID Numbers: FIBRALUNG

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No