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NCT01915511Recruiting

Idiopathic Pulmonary Fibrosis and Interstitial Lung Disease Prospective Outcomes Registry

Duke University

Start Date

6/1/2014

Completion Date

1/1/2031

Summary

The Idiopathic Pulmonary Fibrosis Prospective Outcomes (IPF-PRO) Registry started recruiting in 2014 with the objective of studying Idiopathic Pulmonary Fibrosis. In 2018, the registry expanded to include recruitment of participants with other chronic fibrosing interstitial lung diseases (ILDs) with progressive phenotype also referred to as progressive fibrosing interstitial lung diseases in the Chronic Fibrosis Interstitial Lung Disease with Progressive Phenotype (ILD-PRO) Registry. When the third phase of the registry begins, the IPF-PRO registry will enroll additional patients with idiopathic pulmonary fibrosis. 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 of 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.

Detailed Description

This registry originally enrolled a total of 1002 participants newly diagnosed with IPF and continues to enroll patients with other chronic fibrosing ILDs with newly identified progressive phenotype to reach an enrollment of 1000 patients. Participants will be enrolled in three phases, (IPF-PRO and ILD-PRO) over a span of 8 years at approximately 50 sites experienced in the diagnosis and treatment of ILD in the United States. Enrollment for the original IPF cohort started in 2014 and ended in October 2018, with 1002 total participants enrolled. In the third phase of the registry new enrollment for patients with IPF will restart in 2023-2024 with the plan to enroll up to 1000 new IPF patients, for a total IPF enrollment of 2000. Enrollment for other chronic fibrosing ILDs with newly identified progressive phenotype cohort was initiated in February 2019 and will end when enrollment reaches 1000 participants with the potential of enrolling another 1000 participants with other chronic fibrosing ILDs with newly identified without a progressive phenotype. Data and samples will be collected from participants for approximately 5 years for the IPF cohort. For the chronic fibrosing ILD with progressive phenotype cohort, data and samples will be collected for a minimum of 3 years, up to approximately 5 years. Participant management and treatment decisions will be determined by participants and their health care professionals.

Eligibility Criteria

Age Range: 21 years to No maximum

Inclusion Criteria: * Willing and able to provide informed consent * Established a new diagnosis (within 12 months) of IPF by the enrolling center. * Age 21 years or older, or * 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: * Chronic fibrosing ILD as defined by reticular abnormality with traction bronchiectasis with or without honeycombing confirmed by chest HRCT scan and/or lung biopsy. * 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): * decline in FVC % predicted (% pred) based on ≥10% relative decline * decline in FVC % pred based on ≥5 - \<10% relative decline in FVC combined with worsening of respiratory symptoms as assessed by the site investigator * 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 * decline in DLCO % pred based on≥ 10% relative decline * 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. 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. 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: * Malignancy, treated or untreated, other than skin or early -stage prostate cancer, within the past 5 years * Currently listed for lung transplantation at the time of enrollment * Currently enrolled in an interventional clinical trial at the time of enrollment in this registry * For the additional IPF cohort of 1000 individuals, previous enrollment in this registry.

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Conditions

Idiopathic Pulmonary Fibrosis, Interstitial Lung Disease

Locations

University of Alabama - Birmingham

Birmingham, Alabama 35294

United States

University of Arizona

Tucson, Arizona 85721

United States

University of California - Los Angeles

Los Angeles, California 90024

United States

University of Southern California

Los Angeles, California 90033

United States

Stanford University

Stanford, California 94305

United States

University of Colorado

Aurora, Colorado 80045

United States

Yale University

New Haven, Connecticut 06520

United States

University of Florida

Gainesville, Florida 32610-3175

United States

University of South Florida

Tampa, Florida 33606

United States

Emory University

Atlanta, Georgia 30322

United States

Piedmont Healthcare

Austell, Georgia 30106

United States

University of Chicago

Chicago, Illinois 60637

United States

Northwestern University

Evanston, Illinois 60611

United States

Loyola University Health System

Maywood, Illinois 60153

United States

University of Kansas

Kansas City, Kansas 66160

United States

Tulane University

New Orleans, Louisiana 70112

United States

University of Michigan

Ann Arbor, Michigan 48109

United States

University of Minnesota

Minneapolis, Minnesota 55455

United States

University of Mississippi Medical Center

Jackson, Mississippi 39216

United States

Washington University

St Louis, Missouri 63110

United States

NYU Medical Center

New York, New York 10016

United States

Weill Medical College of Cornell University

New York, New York 10065

United States

UNC Chapel Hill

Chapel Hill, North Carolina 27514

United States

Duke University

Durham, North Carolina 27705

United States

Pulmonix LLC

Greensboro, North Carolina 27403

United States

PMG Research

Wilmington, North Carolina 28401

United States

Wake Forest Baptist Health

Winston-Salem, North Carolina 27157

United States

University of Cincinnati Medical Center

Cincinnati, Ohio 45267

United States

Cleveland Clinic

Cleveland, Ohio 44195

United States

The Ohio State University

Columbus, Ohio 43210

United States

University of Oklahoma

Oklahoma City, Oklahoma 73104

United States

Oregon Clinic

Portland, Oregon 97220

United States

Thomas Jefferson University

Philadelphia, Pennsylvania 19144

United States

Medical University of South Carolina

Charleston, South Carolina 29425

United States

Vanderbilt University

Nashville, Tennessee 37232

United States

University of Texas Southwestern

Dallas, Texas 75235

United States

Baylor University Medical Center at Dallas

Dallas, Texas 75246

United States

Baylor College of Medicine

Houston, Texas 77030

United States

Houston Methodist Lung Center

Houston, Texas 77030

United States

The University of Texas Health Science Center At Houston

Houston, Texas 77030

United States

University of Utah

Salt Lake City, Utah 84108

United States

University from Virginia

Charlottesville, Virginia 22908

United States

Inova

Falls Church, Virginia 22042-3307

United States

The Medical College of Wisconsin

Milwaukee, Wisconsin 53226

United States