Advancing Prevention of Pulmonary Fibrosis

Study Overview
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Brief Summary
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This study plans to learn more about pulmonary fibrosis and how it develops. We want to determine if the disease can be detected early, before the lung is permanently scarred. This study will enroll participants who are not currently diagnosed with pulmonary fibrosis, but who have family members with pulmonary fibrosis. Because there is an increased risk within affected families, this cohort will allow us to learn how pulmonary fibrosis develops, and how the lungs change over time.

Official Title
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Advancing Prevention of Pulmonary Fibrosis - A Cohort Study of Preclinical Pulmonary Fibrosis

Conditions
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IPF, Pulmonary Fibrosis, ILD

Intervention / Treatment
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* Other: Sample collection
* Other: Questionnaires
* Radiation: High resolution CT scan of the chest
* Diagnostic Test: Pulmonary Function Test


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Unaffected first degree relative from Familial Interstitial Pneumonia families (two or more family members with IIP) Age at least 40 years old and younger than 75 years old Exclusion Criteria: Diagnosed with known (physician-diagnosed) pulmonary fibrosis prior to informed consent Other genetic diseases associated with interstitial lung disease Pregnant women

Ages Eligible for Study
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Minimum Age: 40 Years
Maximum Age: 75 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Full Cohort<br>Entire study population | Other: Sample collection<br>* Blood, Urine, Nail, Hair, Saliva, optional Stool<br>Other: Questionnaires<br>* SF-36, Food Frequency, Pulmonary Fibrosis Study questionnaire, COVID questionnaire<br>Radiation: High resolution CT scan of the chest<br>* Upon enrollment, all participants will undergo single prone volumetric thoracic chest CT scan. This will be performed at full inspiration using a 64-slice CT scanner.<br>Diagnostic Test: Pulmonary Function Test<br>* All recruited participants will undergo a pre-bronchodilator spirometry test and a lung diffusion capacity test, both in accordance with ATS guidelines. Exercise capacity (maximum physical exertion) will be assessed by performing a 6-minute walk test, also according to ATS guidelines.<br>|
| Sub-Cohort<br>Randomly selected sub-cohort from the larger group of all participants | Other: Sample collection<br>* Blood, Urine, Nail, Hair, Saliva, optional Stool<br>Other: Questionnaires<br>* SF-36, Food Frequency, Pulmonary Fibrosis Study questionnaire, COVID questionnaire<br>Radiation: High resolution CT scan of the chest<br>* Upon enrollment, all participants will undergo single prone volumetric thoracic chest CT scan. This will be performed at full inspiration using a 64-slice CT scanner.<br>Diagnostic Test: Pulmonary Function Test<br>* All recruited participants will undergo a pre-bronchodilator spirometry test and a lung diffusion capacity test, both in accordance with ATS guidelines. Exercise capacity (maximum physical exertion) will be assessed by performing a 6-minute walk test, also according to ATS guidelines.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Screen all subjects for early Idiopathic Pulmonary Fibrosis (IPF) with High Resolution CT Scan of the Chest | All subjects will be screened with a high resolution CT scan to identify early signs of lung fibrosis. CT scans will be evaluated by study radiologists. | Baseline |