Mechanisms of Familial Pulmonary Fibrosis
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|ClinicalTrials.gov Identifier: NCT03437486|
Recruitment Status : Recruiting
First Posted : February 19, 2018
Last Update Posted : April 5, 2019
|Condition or disease|
|Familial Pulmonary Fibrosis Idiopathic Pulmonary Fibrosis Familial Interstitial Pneumonia|
Potential research subjects will be sent a questionnaire (modified version of the ATS-DLD-78 questionnaire) and study consent form. Individuals with no prior history of lung disease and a dyspnea score of 2 or less will be offered the opportunity to undergo further research evaluation, which will include HRCT scanning, pulmonary function testing (PFTs) and blood draw. Subjects with grade 3 or greater dyspnea or findings of extensive disease on HRCT scan (see below), will be recommended to undergo clinical diagnostic evaluation outside the study. For those subjects that participate in this study, demographic information will be collected and stored in a database, including past medical history, smoking history, medications, and occupational and environmental exposure history.
At 5 year intervals after initial enrollment, subjects without clinical disease will be offered a repeat HRCT scan and PFTs.
Each year after enrollment, the investigators will perform follow-up to ascertain whether subjects have: 1) developed respiratory symptoms consistent with FIP/IPF, 2) undergone additional diagnostic evaluations for lung disease, or 3) begun any new treatments for lung disease. Subjects who have developed respiratory symptoms will be encouraged to seek medical evaluation. For those who have undergone any new diagnostic testing or have been diagnosed with FIP, study coordinators will seek permission to obtain HRCTs, medical records, pulmonary function test results, and lung blocks for evaluation by investigators in this study.
The investigators will use standard criteria established by the ATS/ERS to guide the diagnostic classification of patients who develop FIP. Information will be reviewed by a pathologist, a radiologist, and 3 clinicians. In all cases, the clinicians make the final diagnosis and after reviewing the clinical material (clinical/demographic data and pulmonary physiology), and the radiology and pathology data.
HRCT: A single prone HRCT scan without intravenous contrast will be performed and read by an expert chest radiologist. He will assess the presence, extent, and distribution of areas of ground-glass attenuation, interlobular reticular opacities, irregular thickening of interlobular septa, traction bronchiectasis, and traction bronchiolectasis. The anatomic distribution of each finding will be classified in each lung in one of 4 zones from apex to base (upper, middle, lower, lowest). A score of 0 (absent), 1 (<5%), or 2 (>5% parenchymal involvement) will be given for each descriptor in each lung zone based on visual estimation (total score of 1-16). In addition, HRCT scans will be classified as: 1) normal, 2) abnormal, consistent with early FIP, 3) abnormal, consistent with extensive disease, or 4) abnormal, consistent with other diagnoses. Extensive disease is defined as >5% honeycombing in >2 zones. Other diagnoses could include suspicious lung nodules, extensive emphysema, or other findings requiring clinical referral. Disease progression on HRCT is defined by an increase in the total CT score.
Pulmonary function testing: PFTs will include spirometry, lung volumes, and DLCO.
Specimen collection, processing, and banking: Each subject will have 20 ml blood collected on enrollment and on the day of repeat HRCT. Lymphocytes will be saved for generation of lymphoblastoid cells, DNA isolation, and telomere length analysis. Both serum and plasma will be saved for further studies.
|Study Type :||Observational|
|Estimated Enrollment :||650 participants|
|Official Title:||Mechanisms of Familial Pulmonary Fibrosis|
|Actual Study Start Date :||January 1, 2009|
|Estimated Primary Completion Date :||April 30, 2021|
|Estimated Study Completion Date :||April 30, 2021|
Familial Pulmonary Fibrosis
Subjects asked to participate in this study will be unaffected family members of patients previously diagnosed with familial interstitial pneumonia (FIP) which is the familial form of idiopathic pulmonary fibrosis (IPF).
- clinical diagnosis of interstitial lung disease [ Time Frame: Until study completion of 4-30-2021 ]by ATS/ERS criteria
Biospecimen Retention: Samples With DNA
To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.
Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT03437486
|Contact: Katrina N Douglas, BA||6153433941||Katrina.firstname.lastname@example.org|
|United States, Tennessee|
|Vanderbilt University Medical Center||Recruiting|
|Nashville, Tennessee, United States, 37232|
|Principal Investigator: Timothy Blackwell, MD|
|Principal Investigator:||Timothy Blackwell, MD||Vanderbilt University Medical Center|