Mechanisms of Familial Pulmonary Fibrosis
Trial statusRecruiting
Trial phaseNot listed
Trial typeObservational
Biological sexAll
Age40-75
SponsorVanderbilt University Medical Center
About this trial
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 criteria
This trial does not accept healthy volunteersQualifiers
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
Disqualifiers
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).
Inability to travel to Nashville for 1-2 outpatient visits and/or complete a written or online version of the Interstitial Lung Disease Questionnaire
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.
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.)
Trial population
Inclusion Criteria: 1\) Bloodline members of an affected individual from a family in which two or more members of a family bloodline are known to have had proven Idiopathic Interstitial Pneumonia (IIP) and who have no known diagnosis of IIP or IPF
Trial design
Design model
Family-based
Time perspective
Prospective
Treatments tested in this trial
Not listed
Trial groups
750 Participants
are grouped into 1 trial groupGroup A: Familial Pulmonary Fibrosis
Trial outcomes
Primary outcomes
1
clinical diagnosis of interstitial lung disease
by ATS/ERS criteria
Time frame
Until anticipated study completion of 01-30-2030Sponsors and contacts
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Source ClinicalTrials.gov