An observational study in Interstitial Lung Disease and Idiopathic Pulmonary Fibrosis, sponsored by Columbia University. Completed at 1 site in United States. Open to participants aged 35 Years to 100 Years, including healthy volunteers. Per ClinicalTrials.gov, last updated 2023-11-18.
Sponsored by Columbia University · Observational
The interstitial lung diseases (ILDs) are a family of closely related lung conditions characterized by alveolar inflammation, injury, and fibrosis not due to infection or neoplasia. While previously considered to be rare, a recent nationwide study found that idiopathic pulmonary fibrosis (IPF), a fibrotic ILD with a median survival of only 3.8 years, affects nearly 0.5% of older adults in the U.S. While pirfenidone and nintedanib slow the progression of IPF, neither reverses fibrosis nor prevents progression of the disease,and no studies to date have tested interventions that prevent the development of fibrotic ILDs.
The NHLBI has prioritized research focused on the primary prevention of chronic lung diseases, including ILD. The overall goal of this study is to conduct studies preparatory to and requisite for the testing of ILD preventative interventions.
In the current study, the investigators propose to examine the pulmonary histopathology and biology of early subclinical ILD in healthy adults with a first-degree relative with clinically diagnosed ILD. There are two currently accepted computed tomographic (CT)-based phenotypes of subclinical ILD: high attenuation areas (HAAs) and interstitial lung abnormalities (ILA). Investigators from Columbia University Medical Center have previously shown that HAA has strong construct validity as an imaging biomarker of early subclinical alveolar inflammation and fibrosis among community-dwelling adults using the Multi-Ethnic Study of Atherosclerosis (MESA), an ongoing NHLBI-funded prospective cohort study of 6,814 adults age 45 and older at enrollment in 2000-02. Investigators found that greater HAA at baseline was independently associated with reduced lung function and exercise capacity at 5-year follow-up, exertional dyspnea at 10-year follow-up, and elevated serum levels of matrix metalloproteinase-7 (MMP-7) and interleukin-6 (IL-6). ILA is a distinct qualitative and visually-identified early ILD phenotype on CT that has also shown strong construct validity for ILD. Neither HAA nor ILA has been validated histopathologically.
The lipoprotein substudy will examine the role of high density lipoproteins in patients with ILD. Patients with IPF have previously been shown to have low levels of high density lipoprotein (HDL) and high levels of low density lipoprotein (LDL). Investigators have previously shown that high levels of high-density cholesterol (HDL-C) are associated with a reduction in lung injury, inflammation and fibrosis (subclinical ILD) on CT in community-dwelling adults enrolled in the Multi-Ethnic Study of Atherosclerosis. These data are consistent with animal model data showing that treatment with apolipoprotein A-I (ApoA-I; the main component of HDL) attenuates lung fibrosis. Investigators at Columbia University Medical Center are therefore proposing to examine the associations of HDL and its main components (apolipoprotein A-I, apolipoprotein A-II, and paraoxonase-1) with clinical outcomes (FVC decline, death, lung transplantation and respiratory hospitalizations) and serum biomarkers of lung injury, inflammation and remodeling (SP-A, MMP-7, ICAM-1, IL-1, IL-18) in patients with ILD. Investigators will also explore the structure (using quantitative proteomics) and function (using a macrophage efflux assay and paraoxonase-1 activity assay) of HDL particles in adults with ILD and first-degree family members with subclinical ILD.
Obstructive sleep apnea (OSA) is highly prevalent among adults with interstitial lung disease (ILD) and maybe a risk factor based on our previous studies from MESA (https://www.mesa-nhlbi.org/) and other research studies completed at Columbia University Medical Center. Therefore, the investigators will examine the association between OSA and sub-clinical ILD in at-risk adults.
3,303 studies on the registry are indexed under Lung Diseases; 355 are open to participants now.
This study's enrollment of 125 is below the median of 157 across 929 observational studies indexed under Lung Diseases.
Browse Lung Diseases studies →Columbia University is the lead sponsor of 1,103 studies on the registry; 193 are open to participants now.
Of its 172 completed or terminated interventional studies of FDA-regulated products, 142 (83%) have results posted.
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Adult participants with and without a diagnosis of Interstitial Lung Disease. Adult participants with a diagnosis of interstitial lung disease as per American Thoracic Society (ATS) guidelines. Adult participants with a first-degree relative with a clinical diagnosis of interstitial lung disease. Adult participants who are at least 50 years of age with a smoking history of a minimum of 1 pack per day.
Inclusion Criteria: For "At Risk" participants without clinical ILD
Inclusion Criteria: For "At Risk Smoker" participants without clinical ILD
Exclusion Criteria: For "At-Risk" participants without clinical ILD
Inclusion Criteria: For "Proband" participants with clinical ILD Age 18 years or older
Exclusion Criteria: For "Proband" participants with clinical ILD
There will be no interventions administered to this group, only data collection.
There will be no interventions administered to this group, only data collection
Number of Participants With ILA (Interstitial Lung Abnormalities)
The visual identification of the presence of ILA (Interstitial Lung Abnormalities) on CT chest scan by a thoracic radiologist.
Time frame: During imaging (up to 1 hour)
| Milestone | FAR-ILD Proband Participants | FAR-ILD "At-Risk" Participants |
|---|---|---|
| Started | 27 | 98 |
| Completed | 27 | 98 |
| Not completed | 0 | 0 |
The visual identification of the presence of ILA (Interstitial Lung Abnormalities) on CT chest scan by a thoracic radiologist.
| Participants | FAR-ILD "At Risk" Participants Without ILA | FAR-ILD "At-Risk" Participants With ILA |
|---|---|---|
| Number of Participants With ILA (Interstitial Lung Abnormalities) | 85 | 13 |
Collected over Adverse event data collected through study completion, an average of 1 year.. Non-serious events are listed at a 0% frequency threshold.
| Group | Deaths | Serious | Other |
|---|---|---|---|
| FAR-ILD Proband With an Adverse Event | 1/27 (3.7%) | 1/27 (3.7%) | 0/27 (0%) |
| FAR-ILD "At-Risk" Participant With and Adverse Event | 0/98 (0%) | 0/98 (0%) | 0/98 (0%) |
| Event | FAR-ILD Proband With an Adverse Event | FAR-ILD "At-Risk" Participant With and Adverse Event |
|---|---|---|
| Death due to respiratory causeRespiratory, thoracic and mediastinal disorders | 1/27 | 0/98 |
| Age, Categorical(Participants) | FAR-ILD Proband Participants | FAR-ILD "At-Risk" Participants | Total |
|---|---|---|---|
| <=18 years | 0 | 0 | 0 |
| Between 18 and 65 years | 7 | 89 | 96 |
| >=65 years | 20 | 9 | 29 |
| Age, Continuous(years) | FAR-ILD Proband Participants | FAR-ILD "At-Risk" Participants | Total |
|---|---|---|---|
| Mean | 68 (29 to 87) | 52 (37 to 77) | 54 (29 to 87) |
| Sex: Female, Male(Participants) | FAR-ILD Proband Participants | FAR-ILD "At-Risk" Participants | Total |
|---|---|---|---|
| Female | 10 | 61 | 71 |
| Male | 17 | 37 | 54 |
| Ethnicity (NIH/OMB)(Participants) | FAR-ILD Proband Participants | FAR-ILD "At-Risk" Participants | Total |
|---|---|---|---|
| Hispanic or Latino | 5 | 25 | 30 |
| Not Hispanic or Latino | 22 | 73 | 95 |
| Unknown or Not Reported | 0 | 0 | 0 |
| Race (NIH/OMB)(Participants) | FAR-ILD Proband Participants | FAR-ILD "At-Risk" Participants | Total |
|---|---|---|---|
| American Indian or Alaska Native | 0 | 0 | 0 |
| Asian | 1 | 2 | 3 |
| Native Hawaiian or Other Pacific Islander | 0 | 1 | 1 |
| Black or African American | 1 | 2 | 3 |
| White | 22 | 72 | 94 |
| More than one race | 0 | 0 | 0 |
| Unknown or Not Reported | 3 | 21 | 24 |
| Region of Enrollment(participants) | FAR-ILD Proband Participants | FAR-ILD "At-Risk" Participants | Total |
|---|---|---|---|
| United States | 27 | 98 | 125 |
| Interstitial Lung Abnormality (ILA)(Participants) | FAR-ILD Proband Participants | FAR-ILD "At-Risk" Participants | Total |
|---|---|---|---|
| Count of participants | 27 | 0 | 27 |
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Plan to share: Yes — Researchers will be required to submit a written request to the PI describing the use of the data. The researcher must also document institutional review board (IRB) approval. No identifiable information will be released.
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