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DeciPHer-ILD: A Real-world Patient Registry in Group 3 Pulmonary Hypertension Associated With Interstitial Lung Disease (PH-ILD)
NCT06388421 · United Therapeutics
In plain English
Click the button to translate this study into plain language — what it is, who qualifies, and what participation looks like.
About this study
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
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 design
Enrollment target: 900 participants
Age groups: adult, older_adult
Timeline
Starts: 2025-01-23
Estimated completion: 2030-11-30
Last updated: 2026-04-06
Interventions
Other: Prospective study assessments
Primary outcomes
- • Baseline characteristics as described by demographics, medical history, ILD history, PH history, family history (comorbidities), and diagnostic right heart catheterization values. (Baseline)
Sponsor
United Therapeutics · industry
Contacts & investigators
ContactUnited Therapeutics Global Medical Information · contact · clinicaltrials@unither.com · 919-485-8350
All locations (61)
University of Alabama at BirminghamRecruiting
Birmingham, Alabama, United States
Banner Health - University Medical Center PhoenixRecruiting
Phoenix, Arizona, United States
University of Colorado - Anschutz Medical CampusRecruiting
Aurora, Colorado, United States
National Jewish HealthRecruiting
Denver, Colorado, United States
Pasquerilla Healthcare Center - Georgetown UniversityRecruiting
Washington D.C., District of Columbia, United States
University of FloridaRecruiting
Gainesville, Florida, United States
NCH Research InstituteRecruiting
Naples, Florida, United States
Advent Health Medical Group Advanced Lung DiseaseRecruiting
Orlando, Florida, United States
Intercoastal Medical GroupRecruiting
Sarasota, Florida, United States
Tampa General Hospital/University of South Florida HealthRecruiting
Tampa, Florida, United States
Weston Hospital - Cleveland Clinic FloridaRecruiting
Weston, Florida, United States
Emory UniversityRecruiting
Atlanta, Georgia, United States
Northwestern UniversityRecruiting
Chicago, Illinois, United States
Loyola University Medical CenterRecruiting
Maywood, Illinois, United States
Advocate Christ Medical CenterRecruiting
Oak Lawn, Illinois, United States
Ascension St. VincentRecruiting
Indianapolis, Indiana, United States
University of Kansas Medical CenterRecruiting
Kansas City, Kansas, United States
Norton Pulmonary SpecialistsRecruiting
Louisville, Kentucky, United States
University Medical Center - New OrleansRecruiting
New Orleans, Louisiana, United States
University of Maryland Medical CenterRecruiting
Baltimore, Maryland, United States
Washington University School of MedicineRecruiting
St Louis, Missouri, United States
Morristown Medical CenterRecruiting
Morristown, New Jersey, United States
University of New Mexico Health Sciences CenterRecruiting
Albuquerque, New Mexico, United States
Northwell HealthRecruiting
New Hyde Park, New York, United States
New York University Langone Medical CenterRecruiting
New York, New York, United States
New York Presbyterian Hospital - Weill Cornell MedicineRecruiting
New York, New York, United States
University of North Carolina at Chapel HillRecruiting
Chapel Hill, North Carolina, United States
Duke University School of MedicineRecruiting
Durham, North Carolina, United States
Pulmonix, LLCRecruiting
Greensboro, North Carolina, United States
East Carolina University HealthRecruiting
Greenville, North Carolina, United States
Wakemed Health and HospitalRecruiting
Raleigh, North Carolina, United States
University of Cincinnati Medical CenterRecruiting
Cincinnati, Ohio, United States
The Cleveland ClinicRecruiting
Cleveland, Ohio, United States
Ohio State Richard M. Ross Heart HospitalRecruiting
Columbus, Ohio, United States
The University of Oklahoma Health Sciences CenterRecruiting
Oklahoma City, Oklahoma, United States
Oklahoma Heart InstituteRecruiting
Tulsa, Oklahoma, United States
Summit HealthRecruiting
Bend, Oregon, United States
Legacy Research InstituteRecruiting
Portland, Oregon, United States
Oregon Health & Science UniversityRecruiting
Portland, Oregon, United States
St. Luke's University Health NetworkRecruiting
Bethlehem, Pennsylvania, United States
University of PennsylvaniaRecruiting
Philadelphia, Pennsylvania, United States
Thomas Jefferson University - Sidney Kimmel Medical CollegeRecruiting
Philadelphia, Pennsylvania, United States
Temple Lung CenterRecruiting
Philadelphia, Pennsylvania, United States
Temple University HospitalRecruiting
Philadelphia, Pennsylvania, United States
AnMed HealthRecruiting
Anderson, South Carolina, United States
Medical University of South CarolinaRecruiting
Charleston, South Carolina, United States
StatCare Pulmonary Consultants and Center for Biomedical ResearchRecruiting
Knoxville, Tennessee, United States
Baylor University Medical CenterRecruiting
Dallas, Texas, United States
UT Southwest Medical Center Heart and Lung ClinicRecruiting
Dallas, Texas, United States
Houston MethodistRecruiting
Houston, Texas, United States
University of Texas Health Science Center at HoustonRecruiting
Houston, Texas, United States
Intermountain Medical CenterRecruiting
Murray, Utah, United States
University of Utah HealthRecruiting
Salt Lake City, Utah, United States
Inova Medical GroupRecruiting
Falls Church, Virginia, United States
Pulmonary Associates of Richmond, Inc.Recruiting
Richmond, Virginia, United States
Virginia Commonwealth UniversityRecruiting
Richmond, Virginia, United States
Marshall HealthRecruiting
Huntington, West Virginia, United States
University of Wisconsin Hospital and ClinicsRecruiting
Madison, Wisconsin, United States
Aurora St. Luke's Medical CenterRecruiting
Milwaukee, Wisconsin, United States
Medical College of WisconsinRecruiting
Milwaukee, Wisconsin, United States
Auxilio Mutuo HospitalRecruiting
Guaynabo, Puerto Rico, Puerto Rico