IPF-PRO/ILD-PRO Registry

Currently Recruiting: Participants

Anticipated Sample Size: Two cohorts of 1,000 IPF patients and 1,000 ILD patients

Study Timeline: This study is expected to conclude recruitment in 2029.

ClinicalTrials.gov ID:  NCT01915511

What is the IPF-PRO/ILD-PRO Registry?

This IPF-PRO registry is a prospective registry that will collect information regarding the natural history, health care interactions, participant-reported questionnaire data to assess quality of life, and the methods of treatment of participants with a diagnosis of idiopathic pulmonary fibrosis (IPF) or another chronic fibrosing interstitial lung disease (ILD) with progressive phenotype established at the enrolling centers. In addition, blood samples and chest image studies will be collected and banked for future research projects.

Study Objective

To study the natural history of IPF & non-IPF chronic fibrosing ILD, current practice patterns for diagnosis of IPF & non-IPF chronic fibrosing ILD, the disease impact on patients' quality of life, and the management practices compared to existing guidelines.

Unique Aspects of Project

Both IPF and ILD are rare diseases; the registry is collecting clinical and QoL data as well as biosamples on these unique cohorts of patients.

Inclusion Criteria

1. Willing and able to provide informed consent
2. Established a new diagnosis (within 12 months) of IPF by the enrolling center.
3. Age 21 years or older, or a diagnosis of a non-IPF ILD of any duration, including, but not limited to Idiopathic Non-Specific Interstitial Pneumonia (iNSIP), Unclassifiable Idiopathic Interstitial Pneumonias (IIPs), Interstitial Pneumonia with Autoimmune Features (IPAF), Autoimmune ILDs such as Rheumatoid Arthritis (RA-ILD) and Systemic Sclerosis (SSc-ILD), Chronic Hypersensitivity Pneumonitis (HP), Sarcoidosis or Exposure-related ILDs such as asbestosis with progressive phenotype during the last 24 months by the enrolling center that meets the following criteria:
   1. Chronic fibrosing ILD as defined by a reticular abnormality with traction bronchiectasis with or without honeycombing confirmed by chest HRCT scan and/or lung biopsy.
   2. Progressive phenotype as defined by fulfilling at least one of the criteria below of fibrotic changes (progression set point) within the last 24 months regardless of treatment considered appropriate in individual ILDs (8):
      1. decline in FVC % predicted (% pred) based on ≥10% relative decline
      2. decline in FVC % pred based on ≥5 - <10% relative decline in FVC combined with worsening of respiratory symptoms as assessed by the site investigator
      3. decline in FVC % pred based on ≥5 - <10% relative decline in FVC combined with increasing extent of fibrotic changes on chest imaging (HRCT scan) as assessed by the site investigator
      4. decline in DLCO % pred based on≥ 10% relative decline
      5. worsening of respiratory symptoms as well as increasing extent of fibrotic changes on chest imaging (HRCT scan) as assessed by the site investigator independent of FVC change.
         1. The relative decline for FVC % predicted is calculated using the formula: Relative Decline= (FVC % Pred (Reference)-FVC % Pred (Screening))/(FVC % Pred (Reference))×100%, where FVC % Pred (Reference) is the greatest measurement of FVC % predicted in the 24 months prior to screening and FVC % Pred (Screening) is the measurement of FVC % predicted at screening.
         2. The relative decline for DLCO % predicted is calculated using the formula: Relative Decline= (DLCO % Pred (Reference)-DLCO % Pred (Screening))/(DLCO % Pred (Reference))×100%, Where DLCO % Pred (Reference) is the greatest measurement of DLCO % Pred in the 24 months prior to screening and DLCO % Pred (Screening) is the measurement of DLCO % Pred at screening

Exclusion Criteria

1. Malignancy, treated or untreated, other than skin or early-stage prostate cancer, within the past five years
2. Currently listed for lung transplantation at the time of enrollment
3. Currently enrolled in an interventional clinical trial at the time of enrollment in this registry
4. For the additional IPF cohort of 1000 individuals: previous enrollment in this registry.

Subjects with a new diagnosis of IPF or a non-IPF chronic fibrosing ILD established at the time of enrollment in the registry are eligible for participation in the IPF-PRO/ILD-PRO registry if the participant meets the selection criteria.

Duration of Study Participation

Between three to five years

Study Follow-Up

Participants are followed for three to five years, approximately twice a year.

Alabama

• University of Alabama-Birmingham, Birmingham

Arizona

• St. Joseph’s Hospital, Phoenix

California

• University of California-Los Angeles, Los Angeles
• University of California, Davis, Sacramento
• Stanford University, Stanford

Connecticut

• Yale University School of Medicine, New Haven

Florida

• University of South Florida, Tampa

Georgia

• Piedmont Healthcare, Austell
• Emory University, Atlanta

Illinois

• University of Chicago, Chicago
• Northwestern University, Chicago
• Loyola University Health System, Maywood

Louisiana

• Tulane University, New Orleans

Massachusetts

• Lahey Clinic, Burlington

Michigan

• University of Michigan, Ann Arbor

Minnesota

• University of Minnesota, Minneapolis

Missouri

• Washington University, St. Louis

New York

• Weill Cornell Medical College, New York
• NYU Medical Center, New York
• Albany Medical Center, Albany

North Carolina

• Duke University Medical Center, Durham
• Pulmonix LLC, Greensboro
• UNC Chapel Hill, Chapel Hill
• Salem Chest & Southeastern Clinical Research Center, Winston Salem
• Wilmington Health and PMG Research, Wilmington
• Wake Forest University, Winston-Salem

Ohio

• Cleveland Clinic, Cleveland
• University of Cincinnati Medical Center, Cincinnati

Oklahoma

• University of Oklahoma, Oklahoma City

Oregon

• The Oregon Clinic, PC, Portland

Pennsylvania

• University of Pennsylvania, Philadelphia
• Thomas Jefferson University, Philadelphia

South Carolina

• Medical University of South Carolina, Charleston

Tennessee

• Vanderbilt University Medical Center, Nashville

Texas

• UT Southwestern Medical Center, Dallas
• Baylor University Medical Center at Dallas, Dallas
• Baylor College of Medicine, Houston
• Houston Methodist Lung Center, Houston
• Renovatio Clinical, The Woodlands

Vermont

• University of Vermont Lung Center, Colchester

Virginia

• University of Virginia, Charlottesville

IPF/ILD-Biomarkers

Boehringer Ingelheim

PI: Scott Palmer, MD, MHS

PL: Rosalia Blanco