Clinical Genetics and Screening for Idiopathic Pulmonary Fibrosis

NCT ID: NCT06521125

Last Updated: 2024-07-25

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

NOT_YET_RECRUITING

Total Enrollment

600 participants

Study Classification

OBSERVATIONAL

Study Start Date

2024-09-01

Study Completion Date

2026-09-01

Brief Summary

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Background:

Idiopathic pulmonary fibrosis (IPF) is the most common and severe form of interstitial lung disease. Between 2% and 20% of patients with IPF have a family history of the disease, which is considered the strongest risk factor. Therefore, genetic testing has been increasingly considered as a potential tool to identify patients at risk of developing IPF.

According to some studies, genetic testing (particularly of MUC5B and TERT mutations) could be useful to rapidly identify unidentified and/or asymptomatic individuals (in families as well as in the general population) who have interstitial lung anomalies (ILA) that may indicate a initial stage of pulmonary fibrosis. Finding efficient screening methods and associated targeted treatments for IPF may be essential to improving the prognosis and quality of life of those suffering from this disease.

Objectives of the study:

The study involves two populations of study subjects:

* patients with FPF and sporadic IPF
* first-degree relatives of patients with FPF and sporadic IPF (biological relatives, not spouses)

The primary objective is to determine the prevalence rates of interstitial lung abnormalities in at-risk relatives of patient with IPF and FPF.

Study design:

Multicenter, cross-sectional study without drug and without device conducted in two major Italian tertiary referral hospitals.

The entire project is expected to last 24 months.

Detailed Description

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Conditions

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Familial Pulmonary Fibrosis Idiopathic Pulmonary Fibrosis

Study Design

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Observational Model Type

FAMILY_BASED

Study Time Perspective

CROSS_SECTIONAL

Study Groups

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Patients with FPF and sporadic IPF

High resolution Computed Tomography (HRCT) scans of the Chest

Intervention Type DIAGNOSTIC_TEST

A chest high-resolution computed tomography (HRCT) scan will be performed

Pulmonary Function Testing (PFTs)

Intervention Type DIAGNOSTIC_TEST

Spirometry and diffusing capacity of the lung for carbon monoxide (DLCO) measurements will be performed

Digital lung sounds auscultation

Intervention Type DIAGNOSTIC_TEST

Lung sounds will be recorded using a manual approach with a digital stethoscope

Laboratory Assessments

Intervention Type DIAGNOSTIC_TEST

Clinical laboratory tests will be collected from each participant

DNA sequencing

Intervention Type GENETIC

A sample of genomic DNA from peripheral blood lymphocytes will be collected for DNA sequencing

First-degree relatives of patients with FPF and sporadic IPF

High resolution Computed Tomography (HRCT) scans of the Chest

Intervention Type DIAGNOSTIC_TEST

A chest high-resolution computed tomography (HRCT) scan will be performed

Pulmonary Function Testing (PFTs)

Intervention Type DIAGNOSTIC_TEST

Spirometry and diffusing capacity of the lung for carbon monoxide (DLCO) measurements will be performed

Digital lung sounds auscultation

Intervention Type DIAGNOSTIC_TEST

Lung sounds will be recorded using a manual approach with a digital stethoscope

Laboratory Assessments

Intervention Type DIAGNOSTIC_TEST

Clinical laboratory tests will be collected from each participant

DNA sequencing

Intervention Type GENETIC

A sample of genomic DNA from peripheral blood lymphocytes will be collected for DNA sequencing

Interventions

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High resolution Computed Tomography (HRCT) scans of the Chest

A chest high-resolution computed tomography (HRCT) scan will be performed

Intervention Type DIAGNOSTIC_TEST

Pulmonary Function Testing (PFTs)

Spirometry and diffusing capacity of the lung for carbon monoxide (DLCO) measurements will be performed

Intervention Type DIAGNOSTIC_TEST

Digital lung sounds auscultation

Lung sounds will be recorded using a manual approach with a digital stethoscope

Intervention Type DIAGNOSTIC_TEST

Laboratory Assessments

Clinical laboratory tests will be collected from each participant

Intervention Type DIAGNOSTIC_TEST

DNA sequencing

A sample of genomic DNA from peripheral blood lymphocytes will be collected for DNA sequencing

Intervention Type GENETIC

Eligibility Criteria

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Inclusion Criteria

1. patients aged ≥18 years when signing the informed consent
2. diagnosis of IPF based on 2022 ATS/ERS/JRS/ALAT Guidelines as confirmed by the investigator based on chest HRCT scan and if available surgical lung biopsy
3. diagnosis of FPF defined as the presence of fibrotic ILD in at least two members of the same biological family
4. at least one 1st degree relative \>40 years of age.


a. subjects aged ≥40 years

Exclusion Criteria

1. patients with Interstitial Lung Diseases other than Idiopathic Pulmonary Fibrosis, including but not limited to patients with granulomatous lung disease, autoimmune/collagen vascular disease associated interstitial lung disease, and drug induced interstitial lung disease
2. unwilling or unable to sign informed consent

Criteria for FIRST DEGREE BIOLOGICAL RELATIVES:


1. previous diagnosis of IPF
2. a history of severe or poorly controlled anxiety, severe or poorly controlled depression according to the opinion of the investigators, suicidal ideation, or other psychiatric illness requiring hospitalization
3. unwilling or unable to sign informed consent 400 first-degree relatives of participating patients will be recruited
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

Yes

Sponsors

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Fondazione Policlinico Universitario Agostino Gemelli IRCCS

OTHER

Sponsor Role lead

Responsible Party

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RICHELDI LUCA

Professor

Responsibility Role PRINCIPAL_INVESTIGATOR

Central Contacts

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Luca Richeldi

Role: CONTACT

0630157857

Other Identifiers

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6869

Identifier Type: -

Identifier Source: org_study_id

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