Integrating Molecular, Genomic, Morphology and Environmental Features to Improve Precision Diagnosis and Treatment in Interstitial Lung Diseases (PRECISION-ILD) (PRECISION-ILD)

Integrating Molecular, Genomic, Morphology and Environmental Features to Improve Precision Diagnosis and Treatment in Interstitial Lung Diseases (PRECISION-ILD)

Background: Interstitial Lung Diseases (ILDs) are a heterogeneous group of >100 different, rare diseases, which share the fate of progressive scarring and, ultimately, death. Two anti-fibrotic drugs have demonstrated to slow-down fibrotic progression and steroids/immunosuppressants are commonly used for inflammatory-driven ILDs. However, patient's response to therapeutic options is variable and unpredictable. Similarly, setting a correct diagnosis is difficult in most cases, especially when patients are too sick for invasive procedures.

Objectives: (1) To investigate the differences and commonalities in genetic, genomic and environmental exposures/lifestyle in fibrotic ILDs depending on the entity, disease behavior (progressive fibrosis) and treatment response; (2) To integrate the biomarkers that most impact on prognosis and treatment response in diagnostic algorithms; and (3) To explore the feasibility and cost of implementing a P4 strategy in clinical practice for fibrotic ILDs.

Methods: The investigators will extend, update and unify existing ILD cohorts (Spanish SEPAR ILD Reg, Observatory IPF.cat, CIBERES IPF and Familial ILD cohorts) in whom the researchers will: (1) record demographic, epidemiological, clinical, physiological and lung morphology (radiological +/- histological) information; (2) obtain genetic variation, telomere length, and serum protein markers; (3) investigate environmental exposures (including air-pollution), (4) apply to integrative analytical methods to identify endotypes, predictive biomarkers of disease trajectories, theragnostic biomarkers and new therapeutic targets. Results (5) will be validated in other fibrotic ILD cohorts (e.g.EuILDRegistry, Mexican fibrotic ILD Registry). Besides, the investigators will explore how to translate this P4 medicine approach in clinical practice; (6) implementing a predictive score for prognosis and improving the diagnostic approach through biological data to reduce invasive procedures, and (7) estimate educational requirement and potential health cost implications.

Viability:This project is viable because: (1) cohorts already exist and can be expanded and updated; (2) investigators have ample expertise in translational research and actively participate in ILD consortia; (3) required knowledge and methodology is already in being used by the consortium.

Clinical relevance: Due to the lethality, high social and economic burden of fibrotic ILDs, identifying the best diagnostic and therapeutic approach through preventive, personalized and precise measures is a unique opportunity to improve survival in these patients and efficiency of health-care resources.

Study Overview

• Status: Recruiting
• Conditions: Interstitial Lung Disease; Interstitial Fibrosis
• Intervention / Treatment: Other: Observational register

• Study Type: Observational
• Enrollment (Estimated): 1000

Contacts and Locations

• Study Contact: Name: Maria Molina; Phone Number: 8485 932607500; Email: ufip@bellvitgehospital.cat

Study Locations

• Spain
  • Madrid, Spain
    • Recruiting
    • Hospital La Princesa
    • Contact: Claudia Valenzuela, PI
    • Contact: Phone Number: 915 20 22 00
  • Sevilla, Spain
    • Recruiting
    • Hospital Virgen del Rocío
    • Contact: Jose Antonio Rodriguez Portal, MD, PhD
  • Barcelona
    • Hospitalet de Llobregat, Barcelona, Spain, 08907
      • Recruiting
      • Hospital Universitario de Bellvitge
      • Contact: Jaume Bordas Martinez; Phone Number: 0034 932607689; Email: ufip@bellvitgehospital.cat

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child; Adult; Older Adult
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

Patients older than 18 years with diagnosis of fibrotic ILD of any type in the 12 months prior to inclusion, and radiologic fibrotic changes of at least 5% on chest CT scan.

Description

Inclusion criteria:

• Age > 18 years
• Signed informed consent
• Diagnosis of fibrotic ILD of any type in the 12 months prior to inclusion.
• Radiologic fibrotic changes of at least 5% on chest CT scan
• Ability to comply with the study protocol (in the opinion of the investigator)
• Ability to understand the information given and to sign the informed consent form.

Exclusion Criteria

• Severe, advanced stage or life-limiting chronic diseases prior to the diagnosis of fibrotic ILD, or that represent a high risk of death in the short term (one year after inclusion), such as could be metastatic oncological metastatic diseases, advanced dementia, neurodegenerative diseases in a limiting phase.
• Pregnancy or breastfeeding
• Inability to complete required visits.

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Differences and commonalities in genetic, genomic and environmental exposures/lifestyle in fibrotic ILDs depending on the entity, disease behavior (progressive fibrosis) and treatment response	Record demographic, epidemiological, clinical, physiological and lung morphology (radiological +/- histological) information. Obtain genetic variation, telomere length, and serum protein markers. Investigate environmental exposures (including air-pollution)	3 years
Integration of the biomarkers that most impact on prognosis and treatment response in diagnostic algorithms	Integrative analytical methods to identify endotypes, predictive biomarkers of disease trajectories, theragnostic biomarkers and new therapeutic targets.	3 years
Feasibility and cost of implementing a P4 strategy in clinical practice for fibrotic ILDs	Development of a predictive score for prognosis and improving the diagnostic approach through biological data to reduce invasive procedures, and estimation educational requirement and potential health cost implications.	3 years

Collaborators and Investigators

• Sponsor: Institut d'Investigació Biomèdica de Bellvitge

Study record dates

Study Major Dates

• Study Start (Actual): July 10, 2023
• Primary Completion (Estimated): December 31, 2025
• Study Completion (Estimated): December 31, 2025

Study Registration Dates

• First Submitted: July 21, 2023
• First Submitted That Met QC Criteria: August 16, 2023
• First Posted (Actual): August 21, 2023

Study Record Updates

• Last Update Posted (Actual): August 21, 2023
• Last Update Submitted That Met QC Criteria: August 16, 2023
• Last Verified: August 1, 2023

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Respiratory Tract Diseases; Lung Diseases; Lung Diseases, Interstitial
• Other Study ID Numbers: PMP/00083

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

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