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).
Adults aged 18 years or older
Idiopathic interstitial pneumonia, including idiopathic pulmonary fibrosis
Autoimmune ILD
Chronic hypersensitivity pneumonitis
Confirmed diagnosis of Group 1, 2, 4, or 5 PH
Confirmed diagnosis of Group 3 PH associated with lung diseases and or hypoxia other than fibrotic ILD, as outlined in Inclusion Criterion 2.
Patients receiving inhaled treprostinil (Yutrepia™) at Baseline. Patients receiving other forms of PH therapy will be eligible for enrollment.
Patients with a diagnosis of pulmonary hypertension associated with interstitial lung disease or interstitial lung disease. This includes patients who are and aren't receiving inhaled treprostinil, excluding patients receiving Yutrepia™ at Baseline.
Cohort
Prospective
Prospective study assessments
Other interventionPatients 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.
Changes in clinical features relating to patients' interstitial lung disease and pulmonary hypertension
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