This is a prospective, real world, multicenter, registry of patients with pulmonary hypertension associated with interstitial lung disease (PH-ILD) and interstitial lung disease (ILD).
The purpose of this study is to capture and analyze patient characteristics, treatment patterns, and clinical outcomes of patients with PH-ILD, with the goal of enhancing our current understanding of the disease state and its treatment outcomes. The study also aims to describe the disease trajectory of ILD patients at risk of pulmonary hypertension (PH). In this registry, patients will be enrolled into 1 of 4 cohorts. Cohort 1 will include approximately 200 patients who are not receiving inhaled treprostinil at time of study enrollment. Cohort 2 will include approximately 300 patients who are newly initiated on Tyvaso/Tyvaso DPI. Cohort 3 will include approximately 300 patients who have been receiving Tyvaso/Tyvaso DPI for \>90 days. Cohort 4 will include approximately 100 ILD patients with a prior right heart catheterization (RHC) not meeting the definition of PH. 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; emPHasis-10 Questionnaire; healthcare resource utilization; current medications and rehabilitation; WHO functional class; transplant status; palliative care data; survival data; and RHC.
Study Type
OBSERVATIONAL
Enrollment
900
Patients will complete prospective assessments at 6- and 12-month intervals for up to 5 years. There will be 1 check in at the first 3 months of enrollment. Follow up visits will include a ±45 day visit window. If an assessment is performed within 45 days of the visit window as part of the patient's standard of care, those values will be used in place of a prospective assessment.
Baseline characteristics as described by demographics, medical history, ILD history, PH history, family history (comorbidities), and diagnostic right heart catheterization values.
Time frame: Baseline
Changes in clinical features relating to patients' interstitial lung disease and pulmonary hypertension
Time frame: Followed prospectively for a maximum of 5 years from date of enrollment into the registry
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