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N/A N=125

Families-At-risk for Interstitial Lung Disease Study

Interstitial Lung Disease · Idiopathic Pulmonary Fibrosis

Enrolled (actual)
125
Serious AEs
0.8%
Results posted
Nov 2023
Primary outcome: Primary: Number of Participants With ILA (Interstitial Lung Abnormalities) — 85; 13 Participants

Study Design & Population

Study type
Observational
Phase
N/A
Interventions
Age
Adult, Older Adult · 35+ yrs
Sex
All
Sponsor
Columbia University
Primary completion
Feb 2020

Outcome Measures

OutcomeResultp-value
PRIMARY
Number of Participants With ILA (Interstitial Lung Abnormalities)
85; 13

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. 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.

Eligibility Criteria

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.
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT03641742). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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