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

NCT06388421 | Recruiting

• 1 other identifier: GMS-PH-401
• Study Type: observational
• Target: 900
• Locations: 2 countries
• Sites: 62

Brief 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).

Conditions

Pulmonary Hypertension; Pulmonary Hypertension Due to Lung Diseases and Hypoxia; Interstitial Lung Disease

Keywords

pulmonary hypertension; interstitial lung disease; pulmonary hypertension associated with interstitial lung disease

Outcome Measures

Primary Outcomes (1)

• Baseline characteristics as described by demographics, medical history, ILD history, PH history, family history (comorbidities), and diagnostic right heart catheterization values.

  Baseline

Secondary Outcomes (1)

• Changes in clinical features relating to patients' interstitial lung disease and pulmonary hypertension

  Followed prospectively for a maximum of 5 years from date of enrollment into the registry

Study Arms (4)

Cohort 1

Cohort 1 will include approximately 200 patients who are not receiving inhaled treprostinil at time of study enrollment.

Other: Prospective study assessments

Cohort 2

Cohort 2 will include approximately 300 patients who are newly initiated on Tyvaso/Tyvaso DPI. For patients to be eligible for Cohort 2, they must have initiated Tyvaso/Tyvaso DPI at Baseline or ≤ 90 days prior to Baseline.

Other: Prospective study assessments

Cohort 3

Cohort 3 will include approximately 300 patients who have been receiving Tyvaso/Tyvaso DPI for \> 90 days prior to Baseline.

Other: Prospective study assessments

Cohort 4

Cohort 4 will include approximately 100 ILD patients with a prior RHC not meeting the definition of PH but has a pulmonary artery wedge pressure ≤ 15 mmHg.

Other: Prospective study assessments

Interventions

Prospective study assessments OTHER

Patients will complete prospective assessments at 6- and 12-month intervals for up to 5 years. There will be 1 check in at the first 3 months of enrollment. Follow up visits will include a ±45 day visit window. If an assessment is performed within 45 days of the visit window as part of the patient's standard of care, those values will be used in place of a prospective assessment.

Cohort 1; Cohort 2; Cohort 3; Cohort 4

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Patients with a diagnosis of pulmonary hypertension associated with interstitial lung disease or interstitial lung disease. This includes patients who are and aren't receiving inhaled treprostinil, excluding patients receiving Yutrepia™ at Baseline.

You may qualify if:

• Adults aged 18 years or older
• Diagnosis of fibrotic ILD based on high-resolution computed tomography imaging, including but not limited to:
• Idiopathic interstitial pneumonia, including idiopathic pulmonary fibrosis
• Autoimmune ILD
• Chronic hypersensitivity pneumonitis
• Nonspecific interstitial pneumonia
• Occupational lung disease
• Combined pulmonary fibrosis and emphysema with fibrosis greater than the extent of emphysema on lung imaging as determined by the Investigator
• 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).
• For patients to be eligible for Cohort 1, they must not be receiving inhaled treprostinil at Baseline.
• For patients to be eligible for Cohort 2, they must have initiated
• Tyvaso/Tyvaso DPI at 1 of the following time points:
• Baseline

You may not qualify if:

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

Sponsors & Collaborators

• United Therapeutics: lead

Study Sites (62)

University of Alabama at Birmingham

Birmingham, Alabama, 35294, United States

RECRUITING

Banner Health - University Medical Center Phoenix

Phoenix, Arizona, 85006, United States

RECRUITING

University of Colorado - Anschutz Medical Campus

Aurora, Colorado, 80045, United States

RECRUITING

National Jewish Health

Denver, Colorado, 80206, United States

RECRUITING

Pasquerilla Healthcare Center - Georgetown University

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

RECRUITING

University of Florida

Gainesville, Florida, 32610, United States

RECRUITING

NCH Research Institute

Naples, Florida, 34102, United States

RECRUITING

Advent Health Medical Group Advanced Lung Disease

Orlando, Florida, 32804, United States

RECRUITING

Intercoastal Medical Group

Sarasota, Florida, 34239, United States

RECRUITING

Tampa General Hospital/University of South Florida Health

Tampa, Florida, 33606, United States

RECRUITING

Weston Hospital - Cleveland Clinic Florida

Weston, Florida, 33331, United States

RECRUITING

Emory University

Atlanta, Georgia, 30322, United States

RECRUITING

Northwestern University

Chicago, Illinois, 60611, United States

RECRUITING

Loyola University Medical Center

Maywood, Illinois, 60153, United States

RECRUITING

Advocate Christ Medical Center

Oak Lawn, Illinois, 60453, United States

RECRUITING

Ascension St. Vincent

Indianapolis, Indiana, 46260, United States

RECRUITING

University of Kansas Medical Center

Kansas City, Kansas, 66160, United States

RECRUITING

Norton Pulmonary Specialists

Louisville, Kentucky, 40202, United States

RECRUITING

University Medical Center - New Orleans

New Orleans, Louisiana, 70112, United States

RECRUITING

University of Maryland Medical Center

Baltimore, Maryland, 21201, United States

RECRUITING

Washington University School of Medicine

St Louis, Missouri, 63110, United States

RECRUITING

Dartmouth Hitchcock Medical Center

Lebanon, New Hampshire, 03766, United States

RECRUITING

Morristown Medical Center

Morristown, New Jersey, 07960, United States

RECRUITING

University of New Mexico Health Sciences Center

Albuquerque, New Mexico, 87131, United States

RECRUITING

Northwell Health

New Hyde Park, New York, 11042, United States

RECRUITING

New York University Langone Medical Center

New York, New York, 10016, United States

RECRUITING

New York Presbyterian Hospital - Weill Cornell Medicine

New York, New York, 10021, United States

RECRUITING

University of North Carolina at Chapel Hill

Chapel Hill, North Carolina, 27514, United States

RECRUITING

Duke University School of Medicine

Durham, North Carolina, 27710, United States

RECRUITING

Pulmonix, LLC

Greensboro, North Carolina, 27403, United States

RECRUITING

East Carolina University Health

Greenville, North Carolina, 27834, United States

RECRUITING

Wakemed Health and Hospital

Raleigh, North Carolina, 27610-1231, United States

RECRUITING

University of Cincinnati Medical Center

Cincinnati, Ohio, 45219, United States

RECRUITING

The Cleveland Clinic

Cleveland, Ohio, 44195, United States

RECRUITING

Ohio State Richard M. Ross Heart Hospital

Columbus, Ohio, 43210, United States

RECRUITING

The University of Oklahoma Health Sciences Center

Oklahoma City, Oklahoma, 73104, United States

RECRUITING

Oklahoma Heart Institute

Tulsa, Oklahoma, 74104, United States

RECRUITING

Summit Health

Bend, Oregon, 97701, United States

RECRUITING

Legacy Research Institute

Portland, Oregon, 97210, United States

RECRUITING

Oregon Health & Science University

Portland, Oregon, 97239, United States

RECRUITING

St. Luke's University Health Network

Bethlehem, Pennsylvania, 18015, United States

RECRUITING

University of Pennsylvania

Philadelphia, Pennsylvania, 19104, United States

RECRUITING

Thomas Jefferson University - Sidney Kimmel Medical College

Philadelphia, Pennsylvania, 19107, United States

RECRUITING

Temple Lung Center

Philadelphia, Pennsylvania, 19140, United States

RECRUITING

Temple University Hospital

Philadelphia, Pennsylvania, 19140, United States

RECRUITING

AnMed Health

Anderson, South Carolina, 29621, United States

RECRUITING

Medical University of South Carolina

Charleston, South Carolina, 29425, United States

RECRUITING

StatCare Pulmonary Consultants and Center for Biomedical Research

Knoxville, Tennessee, 37909, United States

RECRUITING

Baylor University Medical Center

Dallas, Texas, 75246, United States

RECRUITING

UT Southwest Medical Center Heart and Lung Clinic

Dallas, Texas, 75390, United States

RECRUITING

Houston Methodist

Houston, Texas, 77030, United States

RECRUITING

University of Texas Health Science Center at Houston

Houston, Texas, 77030, United States

RECRUITING

Intermountain Medical Center

Murray, Utah, 84157, United States

RECRUITING

University of Utah Health

Salt Lake City, Utah, 84108, United States

RECRUITING

Inova Medical Group

Falls Church, Virginia, 22042, United States

RECRUITING

Pulmonary Associates of Richmond, Inc.

Richmond, Virginia, 23230, United States

RECRUITING

Virginia Commonwealth University

Richmond, Virginia, 23298, United States

RECRUITING

Marshall Health

Huntington, West Virginia, 25701, United States

RECRUITING

University of Wisconsin Hospital and Clinics

Madison, Wisconsin, 53792, United States

RECRUITING

Aurora St. Luke's Medical Center

Milwaukee, Wisconsin, 53215, United States

RECRUITING

Medical College of Wisconsin

Milwaukee, Wisconsin, 53226, United States

RECRUITING

Auxilio Mutuo Hospital

Guaynabo, Puerto Rico, 00968, Puerto Rico

RECRUITING

Biospecimen

Retention: SAMPLES WITH DNA

For patients consenting to genomic analysis, a blood sample will be collected. These samples will be shipped to the central laboratory for processing and storage prior to analysis. Genomic analysis will be analyzed for genetic markers that may be associated with clinical response and tolerability. For patients consenting to optional biomarker sample, blood will be collected for the evaluation of biomarkers (specific targets to be determined).

MeSH Terms

Conditions

Hypoxia; Hypertension, Pulmonary; Lung Diseases, Interstitial

Condition Hierarchy (Ancestors)

Signs and Symptoms, Respiratory; Signs and Symptoms; Pathological Conditions, Signs and Symptoms; Lung Diseases; Respiratory Tract Diseases; Hypertension; Vascular Diseases; Cardiovascular Diseases

Central Study Contacts

United Therapeutics Global Medical Information

CONTACT

919-485-8350; clinicaltrials@unither.com

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: INDUSTRY
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: April 12, 2024
• First Posted: April 29, 2024
• Study Start: January 23, 2025
• Primary Completion (Estimated): September 1, 2030
• Study Completion (Estimated): November 30, 2030
• Last Updated: April 29, 2026

Record last verified: 2026-03

Locations

US (61); PR (1)