DeciPHer-ILD: A Real-world Patient Registry in Group 3 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.