Recruiting

Observational Study

Sponsor:

Vanderbilt University Medical Center

Code:

NCT03437486

Conditions

Familial Pulmonary Fibrosis

Idiopathic Pulmonary Fibrosis

Familial Interstitial Pneumonia

Eligibility Criteria

Sex: All

Age: 40 - 70+

Healthy Volunteers: Not accepted

Study Details

Brief 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

Familial Pulmonary Fibrosis

Idiopathic Pulmonary Fibrosis

Familial Interstitial Pneumonia

Study ID

NCT03437486

Start date

Jan 1, 2009

Status verified date

Dec, 2025

Completion date

Jan 30, 2030

Anticipated

Primary completion date

Jan 30, 2030

Anticipated

Eligibility Criteria

Eligibility Criteria

Sex: All

Age: 40 - 70+

Healthy Volunteers: Not accepted

Eligibility Requirements:

1. Bloodline members of an affected individual from a family in which two or more members of a family are known to have Idiopathic Interstitial Pneumonia (IIP) and who have no personal diagnosis of IIP or IPF
2. Sibling or adult child of an affected individual

Exclusion Criteria:

1. Inability to understand the requirements of the study or be unwilling to provide written informed consent (as evidenced by signature on an informed consent document approved by the IRB).
2. Inability to travel to Nashville for 1-2 outpatient visits and/or complete a written or online version of the Interstitial Lung Disease Questionnaire
3. Age < 40 or >75 years old. If the affected relative was younger than 50 years old at the time of IIP diagnosis, potential subjects between age 18 and 40 years may participate when they are up to 10 years younger than the age at relative's diagnosis.
4. Underlying disease with signs and symptoms that could be confused with IIP or IPF symptoms (i.e., rheumatoid arthritis or other connective tissue diseases, occupational lung disease, chemotherapy, etc.)
5. Thought to be unsuitable for participation in the study in the opinion of the investigator

Study Design

Enrollment

750 participants

Anticipated

Interventions and Outcome Measures

Arms

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 and Locations

Central contacts

Tisra H Fadely, BSN, RN

Tisra.h.fadely@vumc.org

Cheryl Markin, BS

cheryl.markin@vumc.org

Locations

Vanderbilt University Medical Center

Recruiting

Nashville, Tennessee, United States, 37232

Principal Investigator:

Margaret Salisbury, MD

More Information

Sponsor

Vanderbilt University Medical Center

Last update posted

Dec 31, 2025

Last verified

Dec, 2025

Trial information was received from ClinicalTrials.gov and was last updated on 2026-09-10. This information was provided to ClinicalTrials.gov by Vanderbilt University Medical Center on 2025-12-31.