Back to Trials
NCT06388421Recruiting

DeciPHer-ILD: A Real-world Patient Registry in Group 3 Pulmonary Hypertension Associated With Interstitial Lung Disease (PH-ILD)

United Therapeutics

Start Date

1/23/2025

Completion Date

11/30/2030

Summary

This is a prospective, real world, multicenter, registry of patients with pulmonary hypertension associated with interstitial lung disease (PH-ILD) and interstitial lung disease (ILD).

Detailed Description

The purpose of this study is to capture and analyze patient characteristics, treatment patterns, and clinical outcomes of patients with PH-ILD, with the goal of enhancing our current understanding of the disease state and its treatment outcomes. The study also aims to describe the disease trajectory of ILD patients at risk of pulmonary hypertension (PH). In this registry, patients will be enrolled into 1 of 4 cohorts. Cohort 1 will include approximately 200 patients who are not receiving inhaled treprostinil at time of study enrollment. Cohort 2 will include approximately 300 patients who are newly initiated on Tyvaso/Tyvaso DPI. Cohort 3 will include approximately 300 patients who have been receiving Tyvaso/Tyvaso DPI for \>90 days. Cohort 4 will include approximately 100 ILD patients with a prior right heart catheterization (RHC) not meeting the definition of PH. As part of the registry, assessments will be completed for data collection in 6- or 12 month intervals, unless the data are already available via standard of care. This patient registry will follow patients for up to 5 years after enrollment. Assessments include pulmonary function tests (PFTs) including diffusing capacity of the lungs for carbon monoxide (DLCO); high resolution computed tomography (HRCT); vital signs; 6-Minute Walk Test; blood draw for plasma N-terminal pro-BNP (NT-proBNP) concentration, genetics analysis, biomarker analysis; echocardiography; EuroQoL 5 Dimension 5 Level Questionnaire (EQ-5D-5L); King's Brief Interstitial Lung Disease Questionnaire (K-BILD); Living with Pulmonary Fibrosis Questionnaire (L-PF); Therapy Administration Questionnaire; emPHasis-10 Questionnaire; healthcare resource utilization; current medications and rehabilitation; WHO functional class; transplant status; palliative care data; survival data; and RHC.

Eligibility Criteria

Age Range: 18 years to No maximum

Inclusion Criteria: 1. Adults aged 18 years or older 2. Diagnosis of fibrotic ILD based on high-resolution computed tomography imaging, including but not limited to: 1. Idiopathic interstitial pneumonia, including idiopathic pulmonary fibrosis 2. Autoimmune ILD 3. Chronic hypersensitivity pneumonitis 4. Nonspecific interstitial pneumonia 5. Occupational lung disease 6. Combined pulmonary fibrosis and emphysema with fibrosis greater than the extent of emphysema on lung imaging as determined by the Investigator 3. For patients to be eligible for Cohorts 1 to 3: RHC confirmed PH (mean pulmonary artery pressure \>20 mmHg, pulmonary artery wedge pressure ≤15 mmHg, pulmonary vascular resistance \>2 WU). 4. For patients to be eligible for Cohort 1, they must not be receiving inhaled treprostinil at Baseline. 5. For patients to be eligible for Cohort 2, they must have initiated Tyvaso/Tyvaso DPI at 1 of the following time points: 1. Baseline 2. ≤90 days prior to Baseline 6. For patients to be eligible for Cohort 3, they must be receiving Tyvaso/Tyvaso DPI at Baseline and for \>90 days prior to Baseline. 7. For patients to be eligible for Cohort 4: Prior RHC not meeting the definition of PH as described in Inclusion Criterion 3 but has a pulmonary artery wedge pressure ≤15 mmHg. 8. Co-enrollment in other observational or interventional studies is permitted. 9. Patient is willing and able to provide informed consent and complete surveys/questionnaires in English or Spanish. Exclusion Criteria: 1. Confirmed diagnosis of Group 1, 2, 4, or 5 PH a. In cases in which a patient has a diagnosis of PH-ILD along with a concomitant diagnosis of another PH group (eg, Group 1), enrollment may be permitted at the Investigator's discretion if, in their clinical judgment, PH-ILD is the predominant driver of the patient's PH. 2. Confirmed diagnosis of Group 3 PH associated with lung diseases and or hypoxia other than fibrotic ILD, as outlined in Inclusion Criterion 2. 3. Patients receiving inhaled treprostinil (Yutrepia™) at Baseline. Patients receiving other forms of PH therapy will be eligible for enrollment.

Interventions

OTHER

Prospective study assessments

Interested in This Trial?

Contact the trial locations directly using the information below to learn more about enrollment.

Expressing interest lets you review the study and consent before we connect you with the research site.

Conditions

Pulmonary Hypertension Due to Lung Diseases and HypoxiaPulmonary HypertensionInterstitial Lung Disease

Locations

University of Alabama at Birmingham

Birmingham, Alabama 35294

United States

Banner Health - University Medical Center Phoenix

Phoenix, Arizona 85006

United States

Cedars-Sinai Medical Center

Los Angeles, California 90048

United States

University of Colorado - Anschutz Medical Campus

Aurora, Colorado 80045

United States

National Jewish Health

Denver, Colorado 80206

United States

Pasquerilla Healthcare Center - Georgetown University

Washington D.C., District of Columbia 20007

United States

University of Florida

Gainesville, Florida 32610

United States

NCH Research Institute

Naples, Florida 34102

United States

Advent Health Medical Group Advanced Lung Disease

Orlando, Florida 32804

United States

Intercoastal Medical Group

Sarasota, Florida 34239

United States

Tampa General Hospital/University of South Florida Health

Tampa, Florida 33606

United States

Weston Hospital - Cleveland Clinic Florida

Weston, Florida 33331

United States

Emory University

Atlanta, Georgia 30322

United States

Northwestern University

Chicago, Illinois 60611

United States

Loyola University Medical Center

Maywood, Illinois 60153

United States

Advocate Christ Medical Center

Oak Lawn, Illinois 60453

United States

Ascension St. Vincent

Indianapolis, Indiana 46260

United States

University of Kansas Medical Center

Kansas City, Kansas 66160

United States

Norton Pulmonary Specialists

Louisville, Kentucky 40202

United States

University Medical Center - New Orleans

New Orleans, Louisiana 70112

United States

University of Maryland Medical Center

Baltimore, Maryland 21201

United States

Washington University School of Medicine

St Louis, Missouri 63110

United States

Dartmouth Hitchcock Medical Center

Lebanon, New Hampshire 03766

United States

Morristown Medical Center

Morristown, New Jersey 07960

United States

HMH Jersey Shore University Medical Ctr.

Neptune City, New Jersey 07753

United States

University of New Mexico Health Sciences Center

Albuquerque, New Mexico 87131

United States

Northwell Health

New Hyde Park, New York 11042

United States

New York University Langone Medical Center

New York, New York 10016

United States

New York Presbyterian Hospital - Weill Cornell Medicine

New York, New York 10021

United States

University of Rochester Medicine

Rochester, New York 14623

United States

University of North Carolina at Chapel Hill

Chapel Hill, North Carolina 27514

United States

Duke University School of Medicine

Durham, North Carolina 27710

United States

Pulmonix, LLC

Greensboro, North Carolina 27403

United States

East Carolina University Health

Greenville, North Carolina 27834

United States

Wakemed Health and Hospital

Raleigh, North Carolina 27610-1231

United States

University of Cincinnati Medical Center

Cincinnati, Ohio 45219

United States

University Hospitals Cleveland Medical Center

Cleveland, Ohio 44106

United States

The Cleveland Clinic

Cleveland, Ohio 44195

United States

Ohio State Richard M. Ross Heart Hospital

Columbus, Ohio 43210

United States

The University of Oklahoma Health Sciences Center

Oklahoma City, Oklahoma 73104

United States

Oklahoma Heart Institute

Tulsa, Oklahoma 74104

United States

Summit Health

Bend, Oregon 97701

United States

Legacy Research Institute

Portland, Oregon 97210

United States

Oregon Health & Science University

Portland, Oregon 97239

United States

St. Luke's University Health Network

Bethlehem, Pennsylvania 18015

United States

University of Pennsylvania

Philadelphia, Pennsylvania 19104

United States

Thomas Jefferson University - Sidney Kimmel Medical College

Philadelphia, Pennsylvania 19107

United States

Temple Lung Center

Philadelphia, Pennsylvania 19140

United States

Temple University Hospital

Philadelphia, Pennsylvania 19140

United States

AnMed Health

Anderson, South Carolina 29621

United States

Medical University of South Carolina

Charleston, South Carolina 29425

United States

StatCare Pulmonary Consultants and Center for Biomedical Research

Knoxville, Tennessee 37909

United States

Baylor University Medical Center

Dallas, Texas 75246

United States

UT Southwest Medical Center Heart and Lung Clinic

Dallas, Texas 75390

United States

Houston Methodist

Houston, Texas 77030

United States

University of Texas Health Science Center at Houston

Houston, Texas 77030

United States

Intermountain Medical Center

Murray, Utah 84157

United States

University of Utah Health

Salt Lake City, Utah 84108

United States

Inova Medical Group

Falls Church, Virginia 22042

United States

Sentara Norfolk General Hospital

Norfolk, Virginia 23507

United States

Pulmonary Associates of Richmond, Inc.

Richmond, Virginia 23230

United States

Virginia Commonwealth University

Richmond, Virginia 23298

United States

Marshall Health

Huntington, West Virginia 25701

United States

University of Wisconsin Hospital and Clinics

Madison, Wisconsin 53792

United States

Aurora St. Luke's Medical Center

Milwaukee, Wisconsin 53215

United States

Medical College of Wisconsin

Milwaukee, Wisconsin 53226

United States

Auxilio Mutuo Hospital

Guaynabo, Puerto Rico 00968

Puerto Rico