Overview
This is a prospective, non-interventional, multicenter, registry of patients with pulmonary hypertension associated with interstitial lung disease (PH-ILD).
Description
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
Inclusion Criteria:
- Adults aged 18 years or older
- Diagnosis of ILD by traditional or HRCT determined by the site/institution that conducted the HRCT
- Patients with connective tissue disease must have a baseline forced vital capacity of <70%
- RHC confirmed PH (mean pulmonary artery pressure >20 mmHg, pulmonary arterial 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
- ≤60 days prior to Baseline
- For patients to be eligible for Cohort 3, they must be receiving Tyvaso/Tyvaso DPI
at Baseline and for >60 days prior to Baseline
- Co-enrollment in other observational or interventional studies is permitted
- Patient is willing and able to provide informed consent and complete surveys/questionnaires in English or Spanish
Exclusion Criteria:
- Confirmed diagnosis of Group 1, 2, 4, or 5 PH
- 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
- Patients receiving Yutrepia (inhaled treprostinil) at Baseline.