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

June 8, 2026 updated by: United Therapeutics
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).

Study Overview

Status

Recruiting

Conditions

Intervention / Treatment

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.

Study Type

Observational

Enrollment (Estimated)

900

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Contact

Study Locations

  • Puerto Rico
    • Puerto Rico
      • Guaynabo, Puerto Rico, Puerto Rico, 00968
        • Recruiting
        • Auxilio Mutuo Hospital
        • Contact:
          • Alvaro Aranda, MD
          • Phone Number: 787-200-4545
  • United States
    • Alabama
      • Birmingham, Alabama, United States, 35294
        • Recruiting
        • University of Alabama at Birmingham
        • Contact:
          • Tejaswini Kulkarni, MD
          • Phone Number: 205-934-3411
    • Arizona
      • Phoenix, Arizona, United States, 85006
        • Recruiting
        • Banner Health - University Medical Center Phoenix
        • Contact:
          • Karim El-Kersh, MD
          • Phone Number: 602-229-2200
    • California
      • Los Angeles, California, United States, 90048
        • Recruiting
        • Cedars-Sinai Medical Center
        • Contact:
          • Yuri Matusov, MD
          • Phone Number: 310-423-8784
    • Colorado
      • Aurora, Colorado, United States, 80045
        • Recruiting
        • University of Colorado - Anschutz Medical Campus
        • Contact:
          • Todd Bull, MD
          • Phone Number: 720-848-5300
      • Denver, Colorado, United States, 80206
        • Recruiting
        • National Jewish Health
        • Contact:
          • Marjorie George, MD
        • Contact:
    • District of Columbia
      • Washington D.C., District of Columbia, United States, 20007
        • Recruiting
        • Pasquerilla Healthcare Center - Georgetown University
        • Contact:
          • Tunay Kuru, MD
          • Phone Number: 202-444-8830
    • Florida
      • Gainesville, Florida, United States, 32610
        • Recruiting
        • University of Florida
        • Contact:
          • Ali Ataya, MD
          • Phone Number: 352-273-8740
      • Naples, Florida, United States, 34102
        • Recruiting
        • NCH Research Institute
        • Contact:
          • David Lindner, DO
          • Phone Number: 239-624-8387
      • Orlando, Florida, United States, 32804
        • Recruiting
        • Advent Health Medical Group Advanced Lung Disease
        • Contact:
          • Melisa Wilson, DNP, APRN, BC
          • Phone Number: 407-303-3638
      • Sarasota, Florida, United States, 34239
        • Recruiting
        • Intercoastal Medical Group
        • Contact:
          • Ryan Dunn, MD
          • Phone Number: 941-379-1799
      • Tampa, Florida, United States, 33606
        • Recruiting
        • Tampa General Hospital/University of South Florida Health
        • Contact:
          • Ricardo Restrepo-Jaramillo, MD
          • Phone Number: 813-821-8038
      • Weston, Florida, United States, 33331
        • Recruiting
        • Weston Hospital - Cleveland Clinic Florida
        • Contact:
          • David Zisman, MD
          • Phone Number: 877-463-2010
    • Georgia
      • Atlanta, Georgia, United States, 30322
        • Recruiting
        • Emory University
        • Contact:
          • Aaron Trammell, MD, MSc
          • Phone Number: 404-778-5736
    • Illinois
      • Chicago, Illinois, United States, 60611
        • Recruiting
        • Northwestern University
        • Contact:
          • Michael Cuttica, MD
          • Phone Number: 312-695-4077
      • Maywood, Illinois, United States, 60153
        • Recruiting
        • Loyola University Medical Center
        • Contact:
          • Daniel Dilling, MD
          • Phone Number: 888-584-7888
      • Oak Lawn, Illinois, United States, 60453
        • Recruiting
        • Advocate Christ Medical Center
        • Contact:
          • Muhyaldeen Dia, MD
          • Phone Number: 708-636-7575
    • Indiana
      • Indianapolis, Indiana, United States, 46260
        • Recruiting
        • Ascension St. Vincent
        • Contact:
          • Sangita Sudharshan, MD
          • Phone Number: 317-338-6666
    • Kansas
      • Kansas City, Kansas, United States, 66160
        • Recruiting
        • University of Kansas Medical Center
        • Contact:
          • Leslie Spikes, MD
          • Phone Number: 913-588-6045
    • Kentucky
      • Louisville, Kentucky, United States, 40202
        • Recruiting
        • Norton Pulmonary Specialists
        • Contact:
          • John McConnell, MD
          • Phone Number: 502-587-8000
    • Louisiana
      • New Orleans, Louisiana, United States, 70112
        • Recruiting
        • University Medical Center - New Orleans
        • Contact:
          • Amita Krishnan, MD
          • Phone Number: 504-568-4634
    • Maryland
      • Baltimore, Maryland, United States, 21201
        • Recruiting
        • University of Maryland Medical Center
        • Contact:
          • Steven Cassady, MD
          • Phone Number: 410-225-8301
    • Missouri
      • St Louis, Missouri, United States, 63110
        • Recruiting
        • Washington University School of Medicine
        • Contact:
          • Robert Case, MD
          • Phone Number: 314-454-8764
    • New Hampshire
      • Lebanon, New Hampshire, United States, 03766
        • Recruiting
        • Dartmouth Hitchcock Medical Center
        • Contact:
          • Jeffrey Stewart, MD
          • Phone Number: 603-650-5533
    • New Jersey
      • Morristown, New Jersey, United States, 07960
        • Recruiting
        • Morristown Medical Center
        • Contact:
          • Abhishek Singh, MD, PhD
          • Phone Number: 973-971-4179
      • Neptune City, New Jersey, United States, 07753
        • Recruiting
        • HMH Jersey Shore University Medical Ctr.
        • Contact:
          • Eric Costanzo, DO
          • Phone Number: 732-775-9075
    • New Mexico
      • Albuquerque, New Mexico, United States, 87131
        • Recruiting
        • University of New Mexico Health Sciences Center
        • Contact:
          • Lana Melendres-Groves, MD
          • Phone Number: 505-272-4751
    • New York
      • New Hyde Park, New York, United States, 11042
        • Recruiting
        • Northwell Health
        • Contact:
          • Arunabh Talwar, MD
          • Phone Number: 516-465-5433
      • New York, New York, United States, 10016
        • Recruiting
        • New York University Langone Medical Center
        • Contact:
          • Roxana Sulica, MD
          • Phone Number: 212-263-1260
      • New York, New York, United States, 10021
        • Recruiting
        • New York Presbyterian Hospital - Weill Cornell Medicine
        • Contact:
          • Robert Kaner, MD
          • Phone Number: 646-962-2333
      • Rochester, New York, United States, 14623
        • Recruiting
        • University of Rochester Medicine
        • Contact:
          • R. James White, MD, PhD
          • Phone Number: 585-275-4861
    • North Carolina
      • Chapel Hill, North Carolina, United States, 27514
        • Recruiting
        • University of North Carolina at Chapel Hill
        • Contact:
          • Hubert Ford, MD
          • Phone Number: 984-974-5703
      • Durham, North Carolina, United States, 27710
        • Recruiting
        • Duke University School of Medicine
        • Contact:
          • Sudarshan Rajagopal, MD, PhD
          • Phone Number: 919-620-5300
      • Greensboro, North Carolina, United States, 27403
        • Recruiting
        • Pulmonix, LLC
        • Contact:
          • Matthew Hunsucker, MD
          • Phone Number: 336-522-8999
      • Greenville, North Carolina, United States, 27834
        • Recruiting
        • East Carolina University Health
        • Contact:
          • Veeranna Maddipati, MD
          • Phone Number: 252-744-1600
      • Raleigh, North Carolina, United States, 27610-1231
        • Recruiting
        • Wakemed Health and Hospital
        • Contact:
          • Kishan Parikh, MD
          • Phone Number: 919-350-8000
    • Ohio
      • Cincinnati, Ohio, United States, 45219
        • Recruiting
        • University of Cincinnati Medical Center
        • Contact:
          • Jean Elwing, MD
          • Phone Number: 513-475-8523
      • Cleveland, Ohio, United States, 44106
        • Recruiting
        • University Hospitals Cleveland Medical Center
        • Contact:
          • Maroun Matta, MD
          • Phone Number: 440-435-3029
      • Cleveland, Ohio, United States, 44195
        • Recruiting
        • The Cleveland Clinic
        • Contact:
          • Alice Goyanes, MD
          • Phone Number: 216-444-6503
      • Columbus, Ohio, United States, 43210
        • Recruiting
        • Ohio State Richard M. Ross Heart Hospital
        • Contact:
          • Elie Homsy, MD
          • Phone Number: 614-293-8000
    • Oklahoma
      • Oklahoma City, Oklahoma, United States, 73104
        • Recruiting
        • The University of Oklahoma Health Sciences Center
        • Contact:
          • Syed Hussain, MD
          • Phone Number: 405-271-6253
      • Tulsa, Oklahoma, United States, 74104
        • Recruiting
        • Oklahoma Heart Institute
        • Contact:
          • Steven Stroud, MD
          • Phone Number: 918-574-9003
    • Oregon
      • Bend, Oregon, United States, 97701
        • Recruiting
        • Summit Health
        • Contact:
          • Jeremy Feldman, MD
          • Phone Number: 541-382-2811
      • Portland, Oregon, United States, 97239
        • Recruiting
        • Oregon Health & Science University
        • Contact:
          • Jeffrey Robinson, MD
          • Phone Number: 503-297-3778
      • Portland, Oregon, United States, 97210
        • Recruiting
        • Legacy Research Institute
        • Contact:
          • Steven Sears, DO
          • Phone Number: 503-413-5702
    • Pennsylvania
      • Bethlehem, Pennsylvania, United States, 18015
        • Recruiting
        • St. Luke's University Health Network
        • Contact:
          • Douglas Corwin, MD
          • Phone Number: 484-526-3890
      • Philadelphia, Pennsylvania, United States, 19104
        • Recruiting
        • University of Pennsylvania
        • Contact:
          • Jason Fritz, MD
          • Phone Number: 215-662-8766
      • Philadelphia, Pennsylvania, United States, 19140
        • Recruiting
        • Temple University Hospital
        • Contact:
          • Ahmed Sadek, MD
          • Phone Number: 800-836-7536
      • Philadelphia, Pennsylvania, United States, 19140
        • Recruiting
        • Temple Lung Center
        • Contact:
          • Sheila Weaver, DO
          • Phone Number: 800-836-7536
      • Philadelphia, Pennsylvania, United States, 19107
        • Recruiting
        • Thomas Jefferson University - Sidney Kimmel Medical College
        • Contact:
          • Gautam George, MD
          • Phone Number: 215-955-6591
    • South Carolina
      • Anderson, South Carolina, United States, 29621
        • Recruiting
        • AnMed Health
        • Contact:
          • Abhijit Raval, MD
          • Phone Number: 864-512-5667
      • Charleston, South Carolina, United States, 29425
        • Recruiting
        • Medical University of South Carolina
        • Contact:
          • Denise Sese, MD
          • Phone Number: 843-792-9200
    • Tennessee
      • Knoxville, Tennessee, United States, 37909
        • Recruiting
        • StatCare Pulmonary Consultants and Center for Biomedical Research
        • Contact:
          • Kunal Gada, MD
          • Phone Number: 865-934-2670
    • Texas
      • Dallas, Texas, United States, 75246
        • Recruiting
        • Baylor University Medical Center
        • Contact:
          • Susan Mathai, MD
          • Phone Number: 214-820-6856
      • Dallas, Texas, United States, 75390
        • Recruiting
        • UT Southwest Medical Center Heart and Lung Clinic
        • Contact:
          • Kelly Chin, MD
          • Phone Number: 214-645-6616
      • Houston, Texas, United States, 77030
        • Recruiting
        • University of Texas Health Science Center at Houston
        • Contact:
          • Rodeo Abrencillo, MD
          • Phone Number: 832-325-7100
      • Houston, Texas, United States, 77030
        • Recruiting
        • Houston Methodist
        • Contact:
          • Meagan Chavarria, NP
          • Phone Number: 713-441-7779
    • Utah
      • Murray, Utah, United States, 84157
        • Recruiting
        • Intermountain Medical Center
        • Contact:
          • Erin Blackstock, MD
          • Phone Number: 801-507-4800
      • Salt Lake City, Utah, United States, 84108
        • Recruiting
        • University of Utah Health
        • Contact:
          • Emily Beck, MD
          • Phone Number: 801-581-2000
    • Virginia
      • Falls Church, Virginia, United States, 22042
        • Recruiting
        • Inova Medical Group
        • Contact:
          • Christopher Thomas, MD
          • Phone Number: 703-776-2986
      • Norfolk, Virginia, United States, 23507
        • Recruiting
        • Sentara Norfolk General Hospital
        • Contact:
          • Michael Eggert, MD
          • Phone Number: 757-388-9138
      • Richmond, Virginia, United States, 23298
        • Recruiting
        • Virginia Commonwealth University
        • Contact:
          • Jared Dyer, DO
          • Phone Number: 804-828-7929
      • Richmond, Virginia, United States, 23230
        • Recruiting
        • Pulmonary Associates of Richmond, Inc.
        • Contact:
          • Shilpa Johri, MD
          • Phone Number: 804-320-4243
    • West Virginia
      • Huntington, West Virginia, United States, 25701
        • Recruiting
        • Marshall Health
        • Contact:
          • Amro Al-Astal, MD
          • Phone Number: 304-691-1000
    • Wisconsin
      • Madison, Wisconsin, United States, 53792
        • Recruiting
        • University of Wisconsin Hospital and Clinics
        • Contact:
          • James Runo, MD
          • Phone Number: 608-263-7203
      • Milwaukee, Wisconsin, United States, 53226
        • Recruiting
        • Medical College of Wisconsin
        • Contact:
          • Kenneth Presberg, MD
          • Phone Number: 414-955-7040
      • Milwaukee, Wisconsin, United States, 53215
        • Recruiting
        • Aurora St. Luke's Medical Center
        • Contact:
          • Eric Roberts, MD
          • Phone Number: 414-646-2438

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

  • Adult
  • Older Adult

Accepts Healthy Volunteers

No

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.

Description

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.

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

Cohorts and Interventions

Group / Cohort
Intervention / Treatment
Cohort 1
Cohort 1 will include approximately 200 patients who are not receiving inhaled treprostinil at time of study enrollment.
Other: Prospective study assessments
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 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
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 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
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 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
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.

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Time Frame
Baseline characteristics as described by demographics, medical history, ILD history, PH history, family history (comorbidities), and diagnostic right heart catheterization values.
Time Frame: Baseline
Baseline

Secondary Outcome Measures

Outcome Measure
Time Frame
Changes in clinical features relating to patients' interstitial lung disease and pulmonary hypertension
Time Frame: Followed prospectively for a maximum of 5 years from date of enrollment into the registry
Followed prospectively for a maximum of 5 years from date of enrollment into the registry

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

United Therapeutics

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

January 23, 2025

Primary Completion (Estimated)

September 1, 2030

Study Completion (Estimated)

November 30, 2030

Study Registration Dates

First Submitted

April 12, 2024

First Submitted That Met QC Criteria

April 24, 2024

First Posted (Actual)

April 29, 2024

Study Record Updates

Last Update Posted (Actual)

June 10, 2026

Last Update Submitted That Met QC Criteria

June 8, 2026

Last Verified

March 1, 2026

More Information

Terms related to this study

Keywords

Additional Relevant MeSH Terms

Other Study ID Numbers

  • GMS-PH-401

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

product manufactured in and exported from the U.S.

Yes

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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