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

Prior treatment

• Confirmed diagnosis of Group 1, 2, 4, or 5 PH
  • 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.
• Patients receiving inhaled treprostinil (Yutrepia™) at Baseline. Patients receiving other forms of PH therapy will be eligible for enrollment.

Other illness

• Confirmed diagnosis of Group 3 PH associated with lung diseases and or hypoxia other than fibrotic ILD, as outlined in Inclusion Criterion 2.

• Adults aged 18 years or older
• Diagnosis of fibrotic ILD based on high-resolution computed tomography imaging, including but not limited to:
  • Idiopathic interstitial pneumonia, including idiopathic pulmonary fibrosis
  • Autoimmune ILD
  • Chronic hypersensitivity pneumonitis
  • Nonspecific interstitial pneumonia
  • Occupational lung disease
  • Combined pulmonary fibrosis and emphysema with fibrosis greater than the extent of emphysema on lung imaging as determined by the Investigator
• 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).
• For patients to be eligible for Cohort 1, they must not be receiving inhaled treprostinil at Baseline.
• For patients to be eligible for Cohort 2, they must have initiated
  • Tyvaso/Tyvaso DPI at 1 of the following time points:
• Baseline
• ≤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.