Long‑Term Safety and Tolerability of Treprostinil Palmitil Inhalation Powder in Patients with Pulmonary Hypertension Associated with Interstitial Lung Disease

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What is this study about?

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.

1 initial visit and baseline assessments

you attend the first study visit after joining the trial and provide written consent to participate.

the study team performs a physical examination, measures vital signs such as blood pressure and heart rate, and records any use of supplemental oxygen.

a 12‑lead electrocardiogram (ecg) is taken to evaluate heart rhythm.

blood samples are collected for laboratory tests, including measurement of NT‑proBNP, a marker that reflects heart stress.

your ability to walk is tested with the six‑minute walk distance (6mwd), which records how far you can walk in six minutes.

lung function is measured with tests called forced vital capacity (fvc) and forced expiratory volume in one second (fev1).

you complete symptom questionnaires, including the lung disease questionnaire (l‑pf) and a general health survey (eq‑5d‑5l).

2 receiving the study medication

you are given treprostinil palmitil inhalation powder for the duration of the study.

each dose contains 1280 µg of the active ingredient and is delivered as an inhalation powder capsule.

the medication is to be inhaled according to the instructions provided by the study staff; the exact frequency (for example, once daily) will be explained at the visit.

3 daily medication administration

you use the inhalation device to take the prescribed dose of treprostinil palmitil at home as instructed.

the medication is taken for up to 104 weeks (two years) unless the study is stopped early for safety reasons.

4 regular follow‑up visits

you return to the study site at scheduled intervals (for example, every 12 weeks) for safety monitoring.

each visit includes repeat measurements of vital signs, oxygen use, and a physical exam.

laboratory tests and a 12‑lead ecg are performed to check for any adverse effects.

the six‑minute walk distance test and lung function tests (fvc and fev1) are repeated to assess any changes in your condition.

blood is drawn again to measure NT‑proBNP levels.

you complete the same symptom questionnaires (l‑pf and eq‑5d‑5l) to track changes in symptoms and quality of life.

5 monitoring for disease worsening

throughout the study you are asked to report any hospitalizations related to heart or lung problems, a noticeable decline in the six‑minute walk distance (15 % or more), new signs of right‑heart failure, or any need for lung transplantation.

these events are recorded as part of the study’s safety and efficacy evaluation.

6 final study visit and study completion

at the end of the study period (up to 104 weeks) you attend a final visit where all assessments performed at baseline and during follow‑up are repeated.

the final data are used to evaluate the long‑term safety and tolerability of treprostinil palmitil inhalation powder in participants with pulmonary hypertension associated with interstitial lung disease.

Who Can Join the Study?

  • You have already finished the earlier research called the lead‑in PH‑ILD TPIP Study INS1009‑311.
  • You are able to read, understand, and sign a written agreement called informed consent, which shows you agree to follow the study’s rules.
  • You agree not to join any other interventional trials or use investigational drugs or devices while you are in this study.
  • You are a male or female who falls within the age limits set by the study (the study includes both adults and children as defined by the researchers).
  • If you are considered a vulnerable participant (for example, a minor or someone who may need extra protection), you still must meet all the other requirements and may need additional permission.

Who Cannot Join the Study?

  • If you have had side effects (called adverse events) that the doctor believes were caused by the study drug and could be risky, you cannot take part.
  • If you are currently taking or are expected to need any medicines approved for pulmonary arterial hypertension (PAH)—such as prostacyclin, prostacyclin‑like drugs, endothelin receptor antagonists, soluble guanylate cyclase stimulators, or any approved treprostinil therapy for PH‑ILD—you cannot join. (Using a class of drugs called phosphodiesterase 5 inhibitors is allowed.)
  • If you are pregnant, breastfeeding, or could become pregnant without using reliable birth control, you cannot join the study.
  • If you have any other medical or mental health condition, or abnormal lab test results, that the doctor thinks could be unsafe, make it hard for you to finish the study, or could affect the study results, you cannot take part.
  • If you have pulmonary hypertension that belongs to WHO Group 1, 2, 4, or 5, or a Group 3 type that is not related to interstitial lung disease (for example, combined pulmonary fibrosis and emphysema), you cannot join.
  • If you have signs of left‑side heart failure (left ventricular failure), a type called HFpEF (heart failure with preserved ejection fraction), or pulmonary hypertension caused by left‑heart problems (post‑capillary PH), you cannot participate.
  • If your blood platelet count is lower than 50,000 per microliter (platelet count <50 × 10³/µL) or you have unexplained problems with blood clotting in repeated tests, you cannot join.
  • If you are known to be allergic (hypersensitivity) to treprostinil, the study drug TPIP, or any ingredients in the formulation (such as mannitol or leucine), you cannot take part.

Where you can join this trial?

Verified and Recommended Sites

No sites found in this category

Verified Sites

Site Name City Country Status
Evangelismos S.A. Athens Greece
IRCCS Humanitas Research Hospital Rozzano Italy
University General Hospital Of Thessaloniki Ahepa Thessaloniki Greece
Hospital Vall d’Hebron Barcelona Spain
Fondazione IRCCS Policlinico San Matteo Pavia Italy
Medical University Of Vienna Vienna Austria
Assistance Publique Hopitaux De Marseille Marseille France
Hospital Clinic De Barcelona Barcelona Spain
Universidade De Santiago De Compostela Santiago De Compostela Spain
Johannes Kepler University Linz Austria
Rheinische Friedrich-Wilhelms-Universitaet Bonn Bonn Germany
Azienda Ospedaliera Universitaria Federico II Di Napoli Naples Italy
General University Hospital Of Patras Patras Greece
Centre Hospitalier Universitaire De Lille Lille France
Klinikum der Universitaet Muenchen AöR Munich Germany
Thoraxklinik Heidelberg gGmbH Heidelberg Germany
Azienda Unita Sanitaria Locale Di Bologna Bologna Italy
Centre Hospitalier Regional Et Universitaire De Brest Brest France
Medizinische Universitaet Innsbruck Innsbruck Austria
Universitaetsklinikum Essen AöR Essen Germany
Azienda Ospedaliero Universitaria Di Modena Modena Italy
Universita Degli Studi Di Roma La Sapienza Rome Italy
Technische Universitaet Dresden Dresden Germany
Katholieke Universiteit te Leuven Leuven Belgium
Universitaetsklinikum Schleswig-Holstein AöR Kiel Germany
Hospital Universitario Puerta De Hierro De Majadahonda Majadahonda Spain
Hospital Universitario 12 De Octubre Madrid Spain
Bellvitge University Hospital L'hospitalet De Llobregat Spain
Centre Hospitalier Regional D’Angers Angers France
Servei De Salut De Les Illes Balears Palma Spain
Fundacion Para La Investigacion Biomedica Del Hospital Universitario La Paz Madrid Spain
University General Hospital Of Heraklion Heraklion Greece
Fondazione IRCCS San Gerardo Dei Tintori Monza Italy
Université Libre de Bruxelles – Hôpital Erasme Brussels Belgium
Hôpitaux Universitaires Strasbourg – Hôpital de Hautepierre STRASBOURG, Alsace France
Azienda Ospedaliero-Universitaria Policlinico G. Rodolico-San Marco Di Catania Catania Italy
Uniwersytecki Szpital Kliniczny W Bialymstoku Bialystok Poland
Institut de Cancérologie Hématologie Universitaire de Saint Etienne (iCHUSE) Saint Priest En Jarez France
Fakultni Thomayerova nemocnice Prague Czechia
Universitätsklinikum des Saarlandes – Homburg/Saar, Klinik für Urologie und Kinderurologie Homburg Germany
Centre Hospitalier Universitaire De Poitiers Poitiers France
Robert Bosch Gesellschaft fuer medizinische Forschung mbH Stuttgart Germany
Azienda Ospedaliero Universitaria Di Sassari Sassari Italy
Multimedica S.p.A. Milan Italy
Centre Hospitalier Universitaire De Bordeaux Bordeaux France
Centre Hospitalier Universitaire De Nice Nice France
Region Midtjylland Aarhus Denmark
Centre Hospitalier Lyon Sud Pierre Benite France
Hopital Beaujon Clichy France

Other Sites

Site Name City Country Status
Krankenhaus Neuwittelsbach Munich Germany
Onassis Cardiac Surgery Center Kallithea Greece
Ajoeagyfe Kbpvjh Gqhqhkz Ghmt Gauting Germany
Hfpfbkwv ob Ingjxzothf Dwwtgonw asx Phhdaeiabd Vvotzl Bvvcqr Tqwbfaddb Timisoara Romania
Hucmyxun Dn Smowd Mufxp Ekgtph Lisbon Portugal
Ewjfl Uxcbbgxssw Eyxzwqgeu Hoxjtido Bucharest Romania
Slvemrgl Cjudwb Db Pvkcossczyovjfpwg Lxea Dmrzaxhu Cluj Napoca Romania
Woumpethda Sdqjtjd Smsdmwsrhjppbaw Ibb Smmqffp Kjiqeyzft Wciwbrcnubqy Suogqyfsmjs Pvqvwgsdd Zqqivs Ovoboj Zviwylytje W Ljzaaysa Lublin Poland
Eajkikjde Irblwuzqy Fck Ccerxktcajyhdx Dlxloihs Aty Tbgealcwxs Targu Mures Romania
Kriigomfe Sokatpr Sabhcltttncobck ia Jcxg Phdnq Iy Cracow Poland
Lrsoszqwvelgmpip Iagytdyyplb Immenhausen Germany
Llibmszsrqu ac Rbgurrnfhtdzk Munich Germany
Sajmucj Kdfhqlpac Dpxaaswila Jshsa Warsaw Poland

Want to learn more about this study or check if you can participate? Contact us.

Trial status

Country Status Recruitment Start
Austria Austria
Not yet recruiting
07.09.2026
Belgium Belgium
Not yet recruiting
07.09.2026
Czechia Czechia
Not yet recruiting
07.09.2026
Denmark Denmark
Not yet recruiting
07.09.2026
France France
Not yet recruiting
07.09.2026
Germany Germany
Not yet recruiting
07.09.2026
Greece Greece
Not yet recruiting
07.09.2026
Italy Italy
Not yet recruiting
07.09.2026
Poland Poland
Not yet recruiting
07.09.2026
Portugal Portugal
Not yet recruiting
07.09.2026
Romania Romania
Not yet recruiting
07.09.2026
Spain Spain
Not yet recruiting
07.09.2026

Trial locations

Investigated Drugs:

Treprostinil Palmitil Inhalation Powder is a medicine that is breathed in as a fine powder. It delivers a drug directly to the lungs where it helps widen the blood vessels that carry blood through the lungs. By doing this, it can lower the pressure in these vessels and make it easier for the heart to pump blood, which can improve breathing and exercise ability for people who have pulmonary hypertension linked to interstitial lung disease. In this study the medicine is being given for a long time to see how safe it is and how well people can tolerate it.

Pulmonary Hypertension associated with Interstitial Lung Disease – It is a condition where the blood pressure in the lung arteries becomes elevated while the lung tissue shows scarring or inflammation. As the lung disease progresses, the pressure in these vessels rises, putting strain on the right side of the heart. This leads to increasing shortness of breath, fatigue, and reduced ability to exercise. Lung function may continue to decline, and oxygen levels can fall. The disease typically worsens gradually, with periods of symptom flare‑ups.

Trial ID:
2025-521769-29-00
Protocol code:
INS1009-312
Trial Phase:
Therapeutic confirmatory (Phase III)

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