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

Last updated: June 8, 2026
Sponsor: United Therapeutics
Overall Status: Active - Recruiting

Phase

N/A

Condition

Chest Pain

Diabetes And Hypertension

Congestive Heart Failure

Treatment

Prospective study assessments

Clinical Study ID

NCT06388421
GMS-PH-401
  • Ages > 18
  • All Genders

Study 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).

Eligibility Criteria

Inclusion

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:

  3. Idiopathic interstitial pneumonia, including idiopathic pulmonary fibrosis

  4. Autoimmune ILD

  5. Chronic hypersensitivity pneumonitis

  6. Nonspecific interstitial pneumonia

  7. Occupational lung disease

  8. Combined pulmonary fibrosis and emphysema with fibrosis greater than the extentof emphysema on lung imaging as determined by the Investigator

  9. For patients to be eligible for Cohorts 1 to 3: RHC confirmed PH (mean pulmonaryartery pressure >20 mmHg, pulmonary artery wedge pressure

≤15 mmHg, pulmonary vascular resistance >2 WU).

  1. For patients to be eligible for Cohort 1, they must not be receiving inhaledtreprostinil at Baseline.

  2. 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 bereceiving Tyvaso/Tyvaso DPI at Baseline and for >90 days prior to Baseline. 7. Forpatients to be eligible for Cohort 4: Prior RHC not meeting the definition of PH asdescribed in Inclusion Criterion 3 but has a pulmonary artery wedge pressure ≤15mmHg. 8. Co-enrollment in other observational or interventional studies ispermitted. 9. Patient is willing and able to provide informed consent and completesurveys/questionnaires in English or Spanish.

Exclusion

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 concomitantdiagnosis of another PH group (eg, Group 1), enrollment may be permitted at theInvestigator's discretion if, in their clinical judgment, PH-ILD is the predominantdriver of the patient's PH.

  2. Confirmed diagnosis of Group 3 PH associated with lung diseases and or hypoxia otherthan fibrotic ILD, as outlined in Inclusion Criterion 2.

  3. Patients receiving inhaled treprostinil (Yutrepia™) at Baseline. Patients receivingother forms of PH therapy will be eligible for enrollment.

Study Design

Total Participants: 900
Treatment Group(s): 1
Primary Treatment: Prospective study assessments
Phase:
Study Start date:
January 23, 2025
Estimated Completion Date:
November 30, 2030

Study 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.

Connect with a study center

  • Auxilio Mutuo Hospital

    Guaynabo, Puerto Rico 00968
    Puerto Rico

    Active - Recruiting

  • University of Alabama at Birmingham

    Birmingham, Alabama 35294
    United States

    Active - Recruiting

  • Banner Health - University Medical Center Phoenix

    Phoenix, Arizona 85006
    United States

    Active - Recruiting

  • Cedars-Sinai Medical Center

    Los Angeles, California 90048
    United States

    Active - Recruiting

  • University of Colorado - Anschutz Medical Campus

    Aurora, Colorado 80045
    United States

    Active - Recruiting

  • National Jewish Health

    Denver, Colorado 80206
    United States

    Active - Recruiting

  • Pasquerilla Healthcare Center - Georgetown University

    Washington D.C., District of Columbia 20007
    United States

    Active - Recruiting

  • University of Florida

    Gainesville, Florida 32610
    United States

    Active - Recruiting

  • NCH Research Institute

    Naples, Florida 34102
    United States

    Active - Recruiting

  • Advent Health Medical Group Advanced Lung Disease

    Orlando, Florida 32804
    United States

    Active - Recruiting

  • Intercoastal Medical Group

    Sarasota, Florida 34239
    United States

    Active - Recruiting

  • Tampa General Hospital/University of South Florida Health

    Tampa, Florida 33606
    United States

    Active - Recruiting

  • Weston Hospital - Cleveland Clinic Florida

    Weston, Florida 33331
    United States

    Active - Recruiting

  • Emory University

    Atlanta, Georgia 30322
    United States

    Active - Recruiting

  • Northwestern University

    Chicago, Illinois 60611
    United States

    Active - Recruiting

  • Loyola University Medical Center

    Maywood, Illinois 60153
    United States

    Active - Recruiting

  • Advocate Christ Medical Center

    Oak Lawn, Illinois 60453
    United States

    Active - Recruiting

  • Ascension St. Vincent

    Indianapolis, Indiana 46260
    United States

    Active - Recruiting

  • University of Kansas Medical Center

    Kansas City, Kansas 66160
    United States

    Active - Recruiting

  • Norton Pulmonary Specialists

    Louisville, Kentucky 40202
    United States

    Active - Recruiting

  • University Medical Center - New Orleans

    New Orleans, Louisiana 70112
    United States

    Active - Recruiting

  • University of Maryland Medical Center

    Baltimore, Maryland 21201
    United States

    Active - Recruiting

  • Washington University School of Medicine

    St Louis, Missouri 63110
    United States

    Active - Recruiting

  • Dartmouth Hitchcock Medical Center

    Lebanon, New Hampshire 03766
    United States

    Active - Recruiting

  • Morristown Medical Center

    Morristown, New Jersey 07960
    United States

    Active - Recruiting

  • HMH Jersey Shore University Medical Ctr.

    Neptune City, New Jersey 07753
    United States

    Active - Recruiting

  • University of New Mexico Health Sciences Center

    Albuquerque, New Mexico 87131
    United States

    Active - Recruiting

  • Northwell Health

    New Hyde Park, New York 11042
    United States

    Active - Recruiting

  • New York Presbyterian Hospital - Weill Cornell Medicine

    New York, New York 10021
    United States

    Active - Recruiting

  • New York University Langone Medical Center

    New York, New York 10016
    United States

    Active - Recruiting

  • University of Rochester Medicine

    Rochester, New York 14623
    United States

    Active - Recruiting

  • New York University Langone Medical Center

    New York 5128581, New York 5128638 10016
    United States

    Active - Recruiting

  • University of North Carolina at Chapel Hill

    Chapel Hill, North Carolina 27514
    United States

    Active - Recruiting

  • Duke University School of Medicine

    Durham, North Carolina 27710
    United States

    Active - Recruiting

  • Pulmonix, LLC

    Greensboro, North Carolina 27403
    United States

    Active - Recruiting

  • East Carolina University Health

    Greenville, North Carolina 27834
    United States

    Active - Recruiting

  • Wakemed Health and Hospital

    Raleigh, North Carolina 27610-1231
    United States

    Active - Recruiting

  • University of Cincinnati Medical Center

    Cincinnati, Ohio 45219
    United States

    Active - Recruiting

  • The Cleveland Clinic

    Cleveland, Ohio 44195
    United States

    Active - Recruiting

  • University Hospitals Cleveland Medical Center

    Cleveland, Ohio 44106
    United States

    Active - Recruiting

  • Ohio State Richard M. Ross Heart Hospital

    Columbus, Ohio 43210
    United States

    Active - Recruiting

  • The University of Oklahoma Health Sciences Center

    Oklahoma City, Oklahoma 73104
    United States

    Active - Recruiting

  • Oklahoma Heart Institute

    Tulsa, Oklahoma 74104
    United States

    Active - Recruiting

  • Summit Health

    Bend, Oregon 97701
    United States

    Active - Recruiting

  • Legacy Research Institute

    Portland, Oregon 97210
    United States

    Active - Recruiting

  • Oregon Health & Science University

    Portland, Oregon 97239
    United States

    Active - Recruiting

  • Oregon Health & Science University

    Portland 5746545, Oregon 5744337 97239
    United States

    Active - Recruiting

  • St. Luke's University Health Network

    Bethlehem, Pennsylvania 18015
    United States

    Active - Recruiting

  • Temple Lung Center

    Philadelphia, Pennsylvania 19140
    United States

    Active - Recruiting

  • Temple University Hospital

    Philadelphia, Pennsylvania 19140
    United States

    Active - Recruiting

  • Thomas Jefferson University - Sidney Kimmel Medical College

    Philadelphia, Pennsylvania 19107
    United States

    Active - Recruiting

  • University of Pennsylvania

    Philadelphia, Pennsylvania 19104
    United States

    Active - Recruiting

  • Temple Lung Center

    Philadelphia 4560349, Pennsylvania 6254927 19140
    United States

    Active - Recruiting

  • Temple University Hospital

    Philadelphia 4560349, Pennsylvania 6254927 19140
    United States

    Active - Recruiting

  • Thomas Jefferson University - Sidney Kimmel Medical College

    Philadelphia 4560349, Pennsylvania 6254927 19107
    United States

    Active - Recruiting

  • AnMed Health

    Anderson, South Carolina 29621
    United States

    Active - Recruiting

  • Medical University of South Carolina

    Charleston, South Carolina 29425
    United States

    Active - Recruiting

  • StatCare Pulmonary Consultants and Center for Biomedical Research

    Knoxville, Tennessee 37909
    United States

    Active - Recruiting

  • Baylor University Medical Center

    Dallas, Texas 75246
    United States

    Active - Recruiting

  • UT Southwest Medical Center Heart and Lung Clinic

    Dallas, Texas 75390
    United States

    Active - Recruiting

  • Houston Methodist

    Houston, Texas 77030
    United States

    Active - Recruiting

  • University of Texas Health Science Center at Houston

    Houston, Texas 77030
    United States

    Active - Recruiting

  • Baylor University Medical Center

    Dallas 4684888, Texas 4736286 75246
    United States

    Active - Recruiting

  • Houston Methodist

    Houston 4699066, Texas 4736286 77030
    United States

    Active - Recruiting

  • Intermountain Medical Center

    Murray, Utah 84157
    United States

    Active - Recruiting

  • University of Utah Health

    Salt Lake City, Utah 84108
    United States

    Active - Recruiting

  • Inova Medical Group

    Falls Church, Virginia 22042
    United States

    Active - Recruiting

  • Sentara Norfolk General Hospital

    Norfolk, Virginia 23507
    United States

    Active - Recruiting

  • Pulmonary Associates of Richmond, Inc.

    Richmond, Virginia 23230
    United States

    Active - Recruiting

  • Virginia Commonwealth University

    Richmond, Virginia 23298
    United States

    Active - Recruiting

  • Pulmonary Associates of Richmond, Inc.

    Richmond 4781708, Virginia 6254928 23230
    United States

    Active - Recruiting

  • Marshall Health

    Huntington, West Virginia 25701
    United States

    Active - Recruiting

  • University of Wisconsin Hospital and Clinics

    Madison, Wisconsin 53792
    United States

    Active - Recruiting

  • Aurora St. Luke's Medical Center

    Milwaukee, Wisconsin 53215
    United States

    Active - Recruiting

  • Medical College of Wisconsin

    Milwaukee, Wisconsin 53226
    United States

    Active - Recruiting

  • Aurora St. Luke's Medical Center

    Milwaukee 5263045, Wisconsin 5279468 53215
    United States

    Active - Recruiting

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