Mechanisms of Familial Pulmonary Fibrosis

Status: Recruiting
Location: See location...
Study Type: Observational
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.

Eligibility
Participation Requirements
Sex: All
Minimum Age: 40
Maximum Age: 75
Healthy Volunteers: f
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⁃ Eligibility Requirements:

• 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

• Sibling or adult child of an affected individual

Locations
United States
Tennessee
Vanderbilt University Medical Center
RECRUITING
Nashville
Contact Information
Primary
Tisra H Fadely, BSN, RN
Tisra.h.fadely@vumc.org
Backup
Cheryl Markin, BS
cheryl.markin@vumc.org
Time Frame
Start Date: 2009-01-01
Estimated Completion Date: 2030-01-30
Participants
Target number of participants: 750
Treatments
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).
Authors
Timothy Blackwell
Sponsors
Leads: Vanderbilt University Medical Center

This content was sourced from clinicaltrials.gov