University of Virginia Natural History Study

A Study of the Natural Progression of Interstitial Lung Disease

Data and specimens will be collected longitudinally from patients seen in the UVA Interstitial Lung Disease (ILD) clinic in order to describe the phenotypic expression of various interstitial lung diseases. Samples will also be collected from a control group for comparison purposes.

All data will be entered into a repository for future research purposes or screening for new studies that become available. This data will help identify trends and hopefully lead to a better understanding of the disease progression, treatment options, and outcomes.

Study Overview

• Status: Enrolling by invitation
• Conditions: Connective Tissue Diseases; Idiopathic Pulmonary Fibrosis; Interstitial Lung Disease; Sarcoidosis; Idiopathic Interstitial Pneumonias; Hypersensitivity Pneumonitis
• Intervention / Treatment: Other: Interstitial Lung Disease

Detailed Description

Following the clinical course of patients with ILD will allow description of the natural history of these diseases and prospective analysis of the following specific questions:

1. Can surrogate physiological markers of disease progression be used in place of mortality? Mortality of a defined cohort of patients will be developed. The surrogate markers include but are not limited to serial cardiopulmonary exercise testing, pulmonary function testing, 6-minute walk testing, HRCT scanning and echocardiography.
2. Do alternative assessments such as quality of life (QOL) questionnaires provide early prediction of physiological change as measured by the previously described parameters? Periodic QOL questionnaires are regularly conducted in clinic and will be followed
3. Does rate of deterioration as measured by these previously described parameters affect response to therapy? If we pre-identify rapid decliners from stable subjects, does this variable affect the response to whatever therapy is applied?
4. Can genetic analysis, genomics, proteomics, microbial and other biomarkers in the blood and cheeks provide insight into the polymorphisms and other elements related to the etiology and pathology of the lung damage?

• Study Type: Observational
• Enrollment (Anticipated): 2500

Contacts and Locations

Study Locations

• United States
  • Virginia
    • Charlottesville, Virginia, United States, 22908
      • University of Virginia Health System

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: Yes
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Patients receiving care in the UVA ILD or Pulmonary clinic

Description

Inclusion Criteria:

• Patient presenting to University of Virginia ILD or Pulmonary clinic
• Family members accompanying patients (as control subjects)

Exclusion Criteria:

• Control subjects cannot have ILD

Study Plan

How is the study designed?

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
ILD Cohort; Patients with Interstitial Lung Disease seen at the UVA ILD or Pulmonary Clinic	Other: Interstitial Lung Disease; Not applicable - this is not an interventional trial
Control Cohort; Control group of patients and family members of those with an Interstitial Lung Disease

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Clinical Course of Disease	Course of disease in patients with ILD will be reviewed prospectively for the duration of the study	Yearly from date of consent until subject is lost to follow-up or death occurs, whichever comes first, assessed up to 144 months
Subject deaths	Causes of death in patients with ILD will be reviewed prospectively for the duration of the study	Yearly from date of consent until subject is lost to follow-up or death occurs, whichever comes first, assessed up to 144 months

Collaborators and Investigators

• Sponsor: University of Virginia
• Investigators: Principal Investigator: Imre Noth, MD, University of Virginia Health System

Study record dates

Study Major Dates

• Study Start (Actual): August 28, 2018
• Primary Completion (Anticipated): August 31, 2035
• Study Completion (Anticipated): August 31, 2035

Study Registration Dates

• First Submitted: April 26, 2021
• First Submitted That Met QC Criteria: May 20, 2021
• First Posted (Actual): May 21, 2021

Study Record Updates

• Last Update Posted (Actual): May 21, 2021
• Last Update Submitted That Met QC Criteria: May 20, 2021
• Last Verified: May 1, 2021

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Infections; Respiratory Tract Infections; Respiratory Tract Diseases; Immune System Diseases; Lymphoproliferative Disorders; Lymphatic Diseases; Hypersensitivity, Immediate; Respiratory Hypersensitivity; Hypersensitivity; Lung Diseases; Pneumonia; Pulmonary Fibrosis; Idiopathic Pulmonary Fibrosis; Connective Tissue Diseases; Lung Diseases, Interstitial; Sarcoidosis; Idiopathic Interstitial Pneumonias; Hamman-Rich Syndrome; Alveolitis, Extrinsic Allergic
• Other Study ID Numbers: 20937

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: YES
• IPD Plan Description: Coded and de-identified data and specimens will be shared with collaborators. MTAs and DUAs are in place for such collaborations. This study also includes a data security plan that includes protective measures for sharing of data between investigators.

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No