Understanding Familial Pulmonary Fibrosis

Mechanisms of Familial Pulmonary Fibrosis

This study follows family members of people with a specific lung disease to see how the disease develops over time and what factors might increase their risk of getting pulmonary fibrosis.

Quick facts

• Study type: Observational
• Enrollment: 750 (estimated)
• Ages: 40 Years to 75 Years
• Sex: All
• Sponsor: Vanderbilt University Medical Center (other)
• Drugs / interventions: chemotherapy
• Locations: 1 site (Nashville, Tennessee)
• Trial ID: NCT03437486 on ClinicalTrials.gov

What this trial studies

This observational study follows first-degree relatives of patients diagnosed with familial interstitial pneumonia to investigate the natural history and risk factors for developing pulmonary fibrosis. Participants will complete yearly questionnaires and undergo evaluations including HRCT scans, pulmonary function tests, and blood draws over a follow-up period of approximately 10 years. The study aims to identify genetic and environmental factors contributing to the disease, particularly in families with a history of idiopathic pulmonary fibrosis.

Who should consider this trial

Good fit: Ideal candidates are first-degree relatives of individuals diagnosed with familial interstitial pneumonia who have no personal history of lung disease.

Not a fit: Patients with existing diagnoses of idiopathic interstitial pneumonia or pulmonary fibrosis will not benefit from this study.

Why it matters

Potential benefit: If successful, this study could lead to better understanding and early detection of pulmonary fibrosis in at-risk individuals.

How similar studies have performed: While similar studies have explored familial patterns in pulmonary fibrosis, this specific longitudinal approach is relatively novel.

Eligibility criteria

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

Where this trial is running

Nashville, Tennessee

• Vanderbilt University Medical Center — Nashville, Tennessee, United States (RECRUITING)

Study contacts

• Principal investigator: Margaret Salisbury, MD — Vanderbilt University Medical Center
• Study coordinator: Tisra H Fadely, BSN, RN

Conditions: Familial Pulmonary Fibrosis, Idiopathic Pulmonary Fibrosis, Familial Interstitial Pneumonia