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

This is a prospective, non-interventional, multicenter, registry of patients with pulmonary hypertension associated with interstitial lung disease (PH-ILD).

The purpose of this study is to observe and capture demographic characteristics, treatment patterns, and clinical outcomes of interest for patients with PH-ILD to further clinical understanding of the epidemiological landscape and outcomes of the disease and treatment.

In this registry, patients will be enrolled into 1 of 3 cohorts. Cohort 1 will include approximately 500 patients who are not receiving inhaled treprostinil at time of study enrollment. Cohort 2 will include approximately 150 patients who are newly initiated on Tyvaso/Tyvaso DPI. Cohort 3 will include approximately 350 patients who have been receiving Tyvaso/Tyvaso DPI for \>60 days.

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; healthcare resource utilization; current medications and rehabilitation; WHO functional class; transplant status; survival data; and RHC.

Eligibility Criteria:
Inclusion Criteria:

1. Adults aged 18 years or older
2. Diagnosis of ILD by traditional or HRCT determined by the site/institution that conducted the HRCT
3. Patients with connective tissue disease must have a baseline forced vital capacity of \<70%
4. RHC confirmed PH (mean pulmonary artery pressure \>20 mmHg, pulmonary arterial wedge pressure ≤15 mmHg, pulmonary vascular resistance \>2 WU).
5. For patients to be eligible for Cohort 1, they must not be receiving inhaled treprostinil at Baseline.
6. 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. ≤60 days prior to Baseline
7. For patients to be eligible for Cohort 3, they must be receiving Tyvaso/Tyvaso DPI at Baseline and for \>60 days prior to Baseline
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
2. Confirmed diagnosis of Group 3 PH related to chronic obstructive pulmonary disease or conditions that cause hypoxemia, such as untreated or inadequately treated obstructive sleep apnea and alveolar hypoventilation disorders
3. Patients receiving Yutrepia (inhaled treprostinil) at Baseline.