Mechanisms of Familial Pulmonary Fibrosis

Part of paid clinical trials in Nashville, Tennessee.

Sponsor
Vanderbilt University Medical Center
Study ID
NCT03437486
Status
Recruiting
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Conditions

  • Familial Interstitial Pneumonia
  • Familial Pulmonary Fibrosis
  • Idiopathic Pulmonary Fibrosis

Eligibility Criteria

Sex
ALL
Age
40 Years - 75 Years
Healthy Volunteers
Not accepted

Study Details

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.

Key Dates

Start date
Jan 1, 2009
Status verified
Dec 2025
Primary completion
Jan 30, 2030
Completion
Jan 30, 2030

Study Design

Enrollment
750 participants (estimated)

Arms

  • Arm: 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).

Primary Outcome Measure

clinical diagnosis of interstitial lung disease [ Time Frame: Until anticipated study completion of 01-30-2030 ]

Central Contacts

Locations (1)

FacilityCityStateZIPSite coordinators
Vanderbilt University Medical CenterNashvilleTennessee37232
Margaret Salisbury, MD (PRINCIPAL_INVESTIGATOR)

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Vanderbilt University Medical Center· Nashville, TN

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