Fibrosing ILD Biomarkers That Rule Acceleration

NCT05635032 | Recruiting

• 1 other identifier: FIBRALUNG
• Study Type: observational
• Target: 150
• Locations: 1 country
• Sites: 1

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.

Conditions

Pulmonary Fibrosis; Sarcoidosis

Outcome Measures

Primary Outcomes (1)

• 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 Outcomes (1)

• Change in microbiome profile in progressive pulmonary fibrosis

  24 months

Other Outcomes (5)

• 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

Study Arms (3)

IPF

Patients with Idiophatic Pulmonary Fibrosis (IPF), serving as a prototype of a progressive fibroproliferative disorder.

Other: Comprehensive clinical assessment and biological samples collection

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

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

Interventions

Comprehensive clinical assessment and biological samples collection OTHER

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.

IPF; Non-Progressive Pulmonary Fibrosis (non-IPF); Progressive Pulmonary Fibrosis (non-IPF)

Eligibility Criteria

• Age: 18 Years - 80 Years
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Adult (18-64), Older Adult (65+)
• 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).

You may qualify if:

• 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

You may not qualify if:

• People who cannot give informed consent
• Pregnancy

Sponsors & Collaborators

• Universidade do Porto: lead
• Centro Hospitalar De São João, E.P.E.: collaborator
• Instituto de Investigação e Inovação em Saúde (i3S): collaborator
• Fundação para a Ciência e a Tecnologia: collaborator

Study Sites (1)

Centro Hospitalar Universitário São João

Porto, Portugal

RECRUITING

Related Links

• Project presentation website

Biospecimen

Retention: SAMPLES WITH DNA

Creation of a biobank of peripheral blood (plasma, serum, DNA, RNA), bronchoalveolar lavage, pharyngeal swabs and lung tissue.

MeSH Terms

Conditions

Pulmonary Fibrosis; Sarcoidosis

Condition Hierarchy (Ancestors)

Lung Diseases, Interstitial; Lung Diseases; Respiratory Tract Diseases; Fibrosis; Pathologic Processes; Pathological Conditions, Signs and Symptoms; Lymphoproliferative Disorders; Lymphatic Diseases; Hemic and Lymphatic Diseases; Hypersensitivity, Delayed; Hypersensitivity; Immune System Diseases

Study Officials

• Helder Novais Bastos, MD, PhD

  Universidade do Porto

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Helder Novais Bastos, MD, PhD

CONTACT

(+351) 220408800; hnovaisbastos@med.up.pt

Janete Santos, PhD

CONTACT

(+351) 225 513 600; investigaclinica@med.up.pt

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: November 22, 2022
• First Posted: December 2, 2022
• Study Start: March 1, 2021
• Primary Completion: February 29, 2024
• Study Completion: March 31, 2026
• Last Updated: October 27, 2023

Record last verified: 2022-11

Locations

PT (1)