Recruiting
DeciPHer-ILD: A Real-world Patient Registry in Group 3 Pulmonary Hypertension Associated With Interstitial Lung Disease (PH-ILD)
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).
ClinicalTrials.gov IDNCT06388421
PhaseNot Applicable
Enrollment900
SponsorUnited Therapeutics
Age18 Years
SexALL
Conditions studied
Pulmonary Hypertension Due to Lung Diseases and Hypoxia, Pulmonary Hypertension, Interstitial Lung Disease
About this study
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).
Interventions
- 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.
Primary outcomes
- Baseline characteristics as described by demographics, medical history, ILD history, PH history, family history (comorbidities), and diagnostic right heart catheterization values. (Baseline)
Eligibility information
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 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
- UC Davis Health Medical Center, Sacramento, California 95817 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
Showing 10 of 71 reported sites. See the official listing for all locations.
Before you participate: Varda Clinical is an independent discovery tool, not the study sponsor or a medical provider. Eligibility can only be determined by the official study team. Discuss potential risks and benefits with a qualified healthcare professional.
Source: ClinicalTrials.gov. Record last refreshed by Varda Clinical: 2026-09-27.