Actively Recruiting
Mechanisms of Familial Pulmonary Fibrosis
Led by Vanderbilt University Medical Center · Updated on 2025-12-31
750
Participants Needed
1
Research Sites
1099 weeks
Total Duration
On this page
AI-Summary
What this Trial Is About
This a prospective, longitudinal study of first-degree family members of patients diagnosed with familial interstitial pneumonia (FIP). FIP is the familial form of idiopathic pulmonary fibrosis (IPF), which is defined as 2 or more bloodline relatives which have a diagnosis of idiopathic interstitial pneumonia (IIP). The most common form of idiopathic interstitial pneumonia in FIP families is IPF (approximately 70%). The inheritance pattern in FIP is consistent with autosomal dominant inheritance with incomplete penetrance. Therefore, individuals in this study have approximately 50% risk of carrying a disease-associated allele. The causative gene is currently only known approximately 20% of families. The main goal of this longitudinal study is to better establish the natural history of FIP and to identify risk factors for later development of symptomatic disease. The investigators' plan is to follow these at-risk individuals with yearly questionnaires and planned in person 2 year follow-ups through age 75 or until they develop symptomatic FIP.
CONDITIONS
Official Title
Mechanisms of Familial Pulmonary Fibrosis
Who Can Participate
Eligibility Criteria
You may qualify if you...
- Be a bloodline member of a family with two or more individuals diagnosed with idiopathic interstitial pneumonia (IIP) and have no personal diagnosis of IIP or IPF
- Be a sibling or adult child of an affected individual
You will not qualify if you...
- Unable to understand study requirements or unwilling to provide informed consent
- Unable to travel to Nashville for 1-2 outpatient visits or unable to complete the Interstitial Lung Disease Questionnaire
- Younger than 40 or older than 75 years old, unless the affected relative was diagnosed before age 50, then participants between 18 and 40 may be eligible
- Have diseases with symptoms that could be confused with IIP or IPF, such as rheumatoid arthritis, connective tissue diseases, occupational lung disease, or chemotherapy effects
- Considered unsuitable for participation by the investigator
AI-Screening
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Trial Site Locations
Total: 1 location
1
Vanderbilt University Medical Center
Nashville, Tennessee, United States, 37232
Actively Recruiting
Research Team
T
Tisra H Fadely, BSN, RN
CONTACT
C
Cheryl Markin, BS
CONTACT
How is the study designed?
Study Type
OBSERVATIONAL
Masking
N/A
Allocation
N/A
Model
N/A
Primary Purpose
N/A
Number of Arms
1
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