Registry for patients with pulmonary hypertension linked to interstitial lung disease

Is this trial a fit for you?

A plain-English reading of the study's rules. Only the study team can confirm whether you qualify.

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.

Why it matters

Who can join: the full rules

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 it's running

• University of Alabama at Birmingham
  Birmingham, Alabama, United States Recruiting
• Banner Health - University Medical Center Phoenix
  Phoenix, Arizona, United States Recruiting
• Cedars-Sinai Medical Center
  Los Angeles, California, United States Recruiting
• UC Davis Health Medical Center
  Sacramento, California, United States Recruiting
• University of Colorado - Anschutz Medical Campus
  Aurora, Colorado, United States Recruiting
• National Jewish Health
  Denver, Colorado, United States Recruiting

+21 more sites. See ClinicalTrials.gov for the full list.

Study contacts

• Study coordinator: United Therapeutics Global Medical Information
• Email: clinicaltrials@unither.com
• Phone: 919-485-8350

Similar studies

Other studies for the same condition or treatment. Recruiting studies are listed first.

• Inhaled mosliciguat for pulmonary hypertension in interstitial lung disease (PH-ILD) patients

  This study tests inhaled mosliciguat versus placebo in people with PH-ILD to see if it helps with their condition.

  Recruiting at 3 sites Phase 3 Pulmovant, Inc. · Kissimmee, Florida and 2 other locations · NCT07816185

• Home exercise for people with pulmonary hypertension and ILD

  A home-based exercise program is tested to see if people with pulmonary hypertension related to interstitial lung disease can improve walking ability and daily function over 16 weeks.

  Recruiting University of Rochester · Rochester, New York · NCT07561034

• Long-Term TPIP Extension for PH-ILD (open-label)

  This study tests whether long-term use of Treprostinil Palmitil Inhalation Powder helps people with pulmonary hypertension due to interstitial lung disease who completed a prior trial.

  Recruiting at 12 sites Phase 3 Insmed Incorporated · Naples, Florida and 11 other locations · NCT07234032

• Once-daily inhaled treprostinil palmitil for pulmonary hypertension linked to interstitial lung disease

  This trial will test whether once-daily inhaled treprostinil palmitil improves exercise ability over 24 weeks in adults with pulmonary hypertension caused by interstitial lung disease.

  Recruiting at 166 sites Phase 3 Insmed Incorporated · Phoenix, Arizona and 185 other locations · NCT07179380

• Using semaglutide to treat advanced lung disease

  This study is testing if a diabetes and weight loss medication called semaglutide can be safely used by adults with advanced lung diseases to see if it helps them manage their weight better.

  Recruiting Phase 1/2 University of Pennsylvania · Philadelphia, Pennsylvania · NCT05746039

• Registry and biorepository for pulmonary hypertension patients

  This study is setting up a collection of health information and blood samples from people with pulmonary hypertension to help researchers learn more about the disease and improve treatments.

  Recruiting University of Kansas Medical Center · Kansas City, Kansas · NCT04808596

How to take part

1. Review the eligibility criteria above with your treating physician.
2. Check the official listing on ClinicalTrials.gov for the latest contact details and recruitment status.
3. Contact the study coordinator or principal investigator to ask for pre-screening. Pre-screening is free and never obligates you to enroll.