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Registry for patients with pulmonary hypertension linked to interstitial lung disease

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

Observational United Therapeutics · NCT06388421

This study is collecting information from patients with pulmonary hypertension linked to interstitial lung disease to see how different treatments affect their health over time.

Quick facts

Study typeObservational
Enrollment900 (estimated)
Ages18 Years and up
SexAll
SponsorUnited Therapeutics Industry-sponsored
Locations62 sites (Birmingham, Alabama and 61 other locations)
Trial IDNCT06388421 on ClinicalTrials.gov

What this trial studies

This observational registry aims to collect and analyze data from patients diagnosed with pulmonary hypertension associated with interstitial lung disease (PH-ILD). It will enroll patients into three cohorts based on their treatment status with Tyvaso/Tyvaso DPI and will follow them for up to five years. Assessments will include pulmonary function tests, imaging, vital signs, and various questionnaires to capture treatment patterns and clinical outcomes. The goal is to enhance understanding of the disease's epidemiology and treatment effects in real-world settings.

Who should consider this trial

Good fit: Ideal candidates include adults aged 18 and older with a confirmed diagnosis of interstitial lung disease and pulmonary hypertension.

Not a fit: Patients who do not have a diagnosis of interstitial lung disease or those who are not eligible for the specified cohorts may not benefit from this registry.

Why it matters

Potential benefit: If successful, this registry could provide valuable insights that lead to improved management and treatment strategies for patients with PH-ILD.

How similar studies have performed: Other observational studies in similar patient populations have shown success in capturing meaningful data, suggesting that this approach is viable.

Eligibility criteria

Show full inclusion / exclusion 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.

Where this trial is running

Birmingham, Alabama and 61 other locations

+12 more sites — see ClinicalTrials.gov for the full list.

Study contacts

How to participate

  1. Review the eligibility criteria above with your treating physician.
  2. Visit the official trial page on ClinicalTrials.gov for the most current contact information and recruitment status.
  3. Contact the listed study coordinator or principal investigator to request pre-screening. Pre-screening is free and never obligates you to enroll.
View on ClinicalTrials.gov → Find more trials like this →
Conditions Pulmonary Hypertension Due to Lung Diseases and HypoxiaPulmonary HypertensionInterstitial Lung Diseasepulmonary hypertensioninterstitial lung diseasepulmonary hypertension associated with interstitial lung disease
Last reviewed 2026-06-13 by the Find a Trial editorial team. Information on this page is for educational purposes and is not medical advice. Always consult qualified healthcare professionals about clinical trial participation.