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Mechanisms of Familial Pulmonary Fibrosis

NCT03437486 · Status: RECRUITING · Type: OBSERVATIONAL · Enrollment: 750

Last updated 2025-12-31

No results posted yet for this study

Summary

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

Sponsors & Collaborators

  • Vanderbilt University Medical Center

    lead OTHER

Principal Investigators

  • Margaret Salisbury, MD · Vanderbilt University Medical Center

Eligibility

Min Age
40 Years
Max Age
75 Years
Sex
ALL
Healthy Volunteers
No

Timeline & Regulatory

Start
2009-01-01
Primary Completion
2030-01-30
Completion
2030-01-30

Countries

  • United States

Study Locations

More Related Trials

Entities

Diseases

Read the full study record

This page highlights key information. For complete eligibility criteria, study locations, investigator contacts, and the full protocol, visit the original record on ClinicalTrials.gov.

View NCT03437486 on ClinicalTrials.gov