The study focuses on Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD), a condition in which high blood pressure in the lungs occurs together with scarring of lung tissue, leading to shortness of breath and reduced ability to exercise. The treatment being examined is an inhaled medication called Treprostinil Palmitil Inhalation Powder, also referred to by the code name TPIP, which is taken as a dry‑powder capsule that is breathed in.
The aim of the study is to evaluate the safety and tolerability of long‑term use of this medication. Participants who have previously taken part in an earlier trial will continue to receive the inhaled powder and will be followed for up to two years. During this time they will attend regular clinic visits where simple checks such as blood tests, heart rhythm monitoring (ECG), measurement of oxygen use, and basic physical examinations are performed. The study also includes a group receiving placebo for comparison.
Throughout the study, participants will undergo a short walking test (6MWD) to see how far they can walk in six minutes, and lung function tests that measure the amount of air they can force out (FVC) and the speed of airflow (FEV1). Blood samples will be taken to check levels of a heart‑stress marker (NT-proBNP). These assessments help researchers understand how the medication affects breathing ability, heart strain, and overall health over the long term.
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