Interstitial Lung DiseaseFAR-ILDNCT03641742
Families-At-risk for Interstitial Lung Disease Study
An observational study of Interstitial Lung Disease and Idiopathic Pulmonary Fibrosis, run by Columbia University at 1 site in United States.
Trial summary
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. Read more about this study
- Who is it for?
- Ages 35 to 100 yearsHealthy volunteers can take part
- Conditions the study is for
- Interstitial Lung Disease, Idiopathic Pulmonary Fibrosis
- How many people
- 125 participants
- Where
- 1 siteUnited States
- Study dates
- 15 Jan 2018to 1 Jul 2023
- Phase
- Observational
- Are there results?
- Yes, posted in the registrySummary in the doctors' view
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 35 to 100 years
- All sexes
- Healthy volunteers accepted
- Adult, Older adult
Study population: 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
- Age 35 years or older, however subjects who are 40 years old and above will undergo HRCT and subjects age 40-65 years old will be eligible to undergo bronchoscopy
- First-degree relative with one of the following clinical diagnoses:
- Idiopathic Pulmonary Fibrosis
- Idiopathic Non-Specific Interstitial Lung Disease (with fibrosis)
- Chronic Hypersensitivity Pneumonitis (with fibrosis)
- Unclassifiable Idiopathic Interstitial Pneumonia (with fibrosis)
- Patients with any ILD characterized by fibrosis on CT chest scan
- Ability to provide informed consent
Inclusion Criteria: For "At Risk Smoker" participants without clinical ILD
- At least 50 years of age
- Smoked at least 1 pack a day for 30 years
Exclusion Criteria: For "At-Risk" participants without clinical ILD
- Known history of interstitial lung disease
- History of illicit drug use within the past year.
- Lower respiratory tract infection in the past 90 days.
- History of chest CT scan in the past year.
- Known history of heart failure or chronic kidney or liver disease.
- Pregnancy or Lactation
Inclusion Criteria: For "Proband" participants with clinical ILD Age 18 years or older
- Has one of the following clinical diagnoses as per ATS guidelines:
- Idiopathic Pulmonary Fibrosis
- Idiopathic Non-Specific Interstitial Lung Disease (with fibrosis)
- Chronic Hypersensitivity Pneumonitis (with fibrosis)
- Unclassifiable Idiopathic Interstitial Pneumonia (with fibrosis)
- Patient with any ILD characterized by fibrosis on CT chest scan
- Ability to provide informed consent
Exclusion Criteria: For "Proband" participants with clinical ILD
- No Living 1st degree relatives.
Trial locations and contacts
1 site in 1 country.
United States1 site
Columbia University Irving Medical Center
New York, New York, 10032
About this study
Official title: The Families-At-risk for Interstitial Lung Disease Study
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.
Read the detailed description
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.
- Keywords
- Interstitial Lung Disease, Pulmonary Fibrosis
- Medical subject headings
- Lung Diseases, Interstitial, Idiopathic Pulmonary Fibrosis, Pulmonary Fibrosis
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
| Study group | Role | What they receive |
|---|---|---|
| FAR-ILD Proband Participants | There will be no interventions administered to this group, only data collection. | |
| FAR-ILD "At-Risk" Participants | There will be no interventions administered to this group, only data collection |
What is being measured
The primary outcome is the main question the study is designed to answer.
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.
Measured: During imaging (up to 1 hour)
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted22 Aug 2018
- Study start15 Jan 2018
- Primary completion11 Feb 2020
- Study completion1 Jul 2023
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Posted resultsParticipant flow, outcome measures and adverse events reported by the sponsor.
- Publications (5)Papers the sponsor has linked to this study.
- Other trials for Interstitial Lung DiseaseStudies of the same condition, open and under way.
- More trials run by Columbia UniversityThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Prospective
- Enrolment
- 125 participants (actual)
Sponsor and investigators
- Lead sponsor
- Columbia University
- Sponsor type
- University, hospital or other
- Collaborators
- University of Washington, Weill Medical College of Cornell University, University of Iowa, National Heart, Lung, and Blood Institute (NHLBI)
- Responsible party
- Principal investigator, Christine Garcia, Columbia University
- Investigators
- Christine Garcia, MD, PhD, Principal investigator, Columbia University
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT03641742
- Sponsor's protocol ID
- AAAR1916
- Other IDs
- 2R01HL103676-05
- Acronym
- FAR-ILD
- Status last verified
- Oct 2023
- First submitted
- 20 Aug 2018
- Last updated
- 18 Nov 2023
- Results posted
- Yes, on ClinicalTrials.gov
- Data monitoring committee
- Yes
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- 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.
Publications
Ost DE, Ernst A, Lei X, Kovitz KL, Benzaquen S, Diaz-Mendoza J, Greenhill S, Toth J, Feller-Kopman D, Puchalski J, Baram D, Karunakara R, Jimenez CA, Filner JJ, Morice RC, Eapen GA, Michaud GC, Estrada-Y-Martin RM, Rafeq S, Grosu HB, Ray C, Gilbert CR, Yarmus LB, Simoff M; AQuIRE Bronchoscopy Registry. Diagnostic Yield and Complications of Bronchoscopy for Peripheral Lung Lesions. Results of the AQuIRE Registry. Am J Respir Crit Care Med. 2016 Jan 1;193(1):68-77. doi: 10.1164/rccm.201507-1332OC.
PubMed 26367186Facciolongo N, Patelli M, Gasparini S, Lazzari Agli L, Salio M, Simonassi C, Del Prato B, Zanoni P. Incidence of complications in bronchoscopy. Multicentre prospective study of 20,986 bronchoscopies. Monaldi Arch Chest Dis. 2009 Mar;71(1):8-14. doi: 10.4081/monaldi.2009.370.
PubMed 19522159Pue CA, Pacht ER. Complications of fiberoptic bronchoscopy at a university hospital. Chest. 1995 Feb;107(2):430-2. doi: 10.1378/chest.107.2.430.
PubMed 7842773Jin F, Mu D, Chu D, Fu E, Xie Y, Liu T. Severe complications of bronchoscopy. Respiration. 2008;76(4):429-33. doi: 10.1159/000151656. Epub 2008 Aug 21.
PubMed 18716395Rosenthal E. New York seeks to tighten rules on medical research. N Y Times Web. 1996 Sep 27:B4. No abstract available.
PubMed 11648036
Study at a glance
From the registry record, last updated by the sponsor on 18 Nov 2023. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- The Families-At-risk for Interstitial Lung Disease Study
- ClinicalTrials.gov ID
- NCT03641742
- Acronym / sponsor ID
- FAR-ILD / AAAR1916
- Keywords
- Interstitial Lung Disease, Pulmonary Fibrosis
- Study type
- Observational
- Recruitment status
- Completed
- Enrolment
- 125 participants (actual)
- Lead sponsor
- Columbia UniversityUniversity, hospital or other
- Collaborators
- University of Washington, Weill Medical College of Cornell University, University of Iowa, National Heart, Lung, and Blood Institute (NHLBI)
- Study start
- 15 Jan 2018 (actual)
- Primary completion
- 11 Feb 2020 (actual)
- Study completion
- 1 Jul 2023 (actual)
- First posted
- 22 Aug 2018 (actual)
- Results
- Posted 18 Nov 2023
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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.
Detailed description
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.
Study design
- Observational model
- Cohort
- Time perspective
- Prospective
- Number of arms
- 2
| Arm | Type | Receives |
|---|---|---|
| FAR-ILD Proband Participants | – | –There will be no interventions administered to this group, only data collection. |
| FAR-ILD "At-Risk" Participants | – | –There will be no interventions administered to this group, only data collection |
Who may qualify
- Ages
- 35 to 100 years
- Sex
- All
- Healthy volunteers
- Accepted
- Age groups
- Adult, Older adult
- Study population
- 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.
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
Number of Participants With ILA (Interstitial Lung Abnormalities)
Time frame: During imaging (up to 1 hour)
The visual identification of the presence of ILA (Interstitial Lung Abnormalities) on CT chest scan by a thoracic radiologist.
Results and safety
Loading the posted results from ClinicalTrials.gov…
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 18 Nov 2023.
- Posted resultsParticipant flow, baseline, all outcome measures and adverse events.
- Background: PubMed 26367186Ost DE, Ernst A, Lei X, Kovitz KL, Benzaquen S, Diaz-Mendoza J, Greenhill S, Toth J, Feller-Kopman D, Puchalski J, Baram D, Karunakara R, Jimenez CA, Filner JJ, Morice RC, Eapen GA, Michaud GC, Estrada-Y-Martin RM, Rafeq S, Grosu HB, Ray C, Gilbert CR, Yarmus LB, Simoff M; AQuIRE Bronchoscopy Registry. Diagnostic Yield and Complications of Bronchoscopy for Peripheral Lung Lesions. Results of the AQuIRE Registry. Am J Respir Crit Care Med. 2016 Jan 1;193(1):68-77. doi: 10.1164/rccm.201507-1332OC.
- Background: PubMed 19522159Facciolongo N, Patelli M, Gasparini S, Lazzari Agli L, Salio M, Simonassi C, Del Prato B, Zanoni P. Incidence of complications in bronchoscopy. Multicentre prospective study of 20,986 bronchoscopies. Monaldi Arch Chest Dis. 2009 Mar;71(1):8-14. doi: 10.4081/monaldi.2009.370.
- Background: PubMed 7842773Pue CA, Pacht ER. Complications of fiberoptic bronchoscopy at a university hospital. Chest. 1995 Feb;107(2):430-2. doi: 10.1378/chest.107.2.430.
- Background: PubMed 18716395Jin F, Mu D, Chu D, Fu E, Xie Y, Liu T. Severe complications of bronchoscopy. Respiration. 2008;76(4):429-33. doi: 10.1159/000151656. Epub 2008 Aug 21.
- Background: PubMed 11648036Rosenthal E. New York seeks to tighten rules on medical research. N Y Times Web. 1996 Sep 27:B4. No abstract available.
- Individual participant dataSharing planned: 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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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
Christine Garcia, MD, PhD principal investigator
Columbia University
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Interstitial Lung Disease and Idiopathic Pulmonary Fibrosis, run by Columbia University at 1 site in United States.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: finished, as of the sponsor's last update on 18 Nov 2023. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in United States. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
Yes. Results are posted in the registry (first posted 18 Nov 2023). A summary is in the For doctors view, and the full results are on ClinicalTrials.gov.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Number of Participants With ILA (Interstitial Lung Abnormalities) (time frame: During imaging (up to 1 hour)). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT03641742, which also lists 5 linked publications.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 18 Nov 2023.