{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T05:46:29Z","timestamp":1793166389740,"version":"4.5.0"},"reference-count":42,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2026,6,15]],"date-time":"2026-06-15T00:00:00Z","timestamp":1781481600000},"content-version":"vor","delay-in-days":75,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pulm. circ."],"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Treprostinil palmitil (TP) inhalation powder (TPIP), an investigational formulation of the treprostinil prodrug TP, was designed to provide prolonged pulmonary vasodilation with once\u2010daily administration. In this phase 2 study (NCT05176951), adults with pulmonary hypertension associated with interstitial lung disease (PH\u2010ILD) were randomized 3:1 to receive TPIP (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200929) or placebo (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200910) for 16 weeks. TPIP was up\u2010titrated from 80\u2009\u00b5g to each patient's maximum tolerated dose (\u2264\u2009640\u2009\u00b5g). Study endpoints were safety and tolerability (primary); pharmacokinetics of TP and treprostinil (secondary); and clinical worsening, change from baseline in 6\u2010min walk distance (6MWD), and N\u2010terminal pro\u2010B\u2010type natriuretic peptide (NT\u2010proBNP) levels (exploratory). Maximum TPIP dose (640\u2009\u00b5g) once daily was achieved by 79.3% of patients. TPIP was well tolerated in this study, with a safety profile generally consistent with previously reported route\u2010specific treatment\u2010emergent adverse events (TEAEs) of inhaled treprostinil, such as cough and dyspnea. TEAEs occurred in 93.1% and 90.0% of patients on TPIP and placebo, respectively. Most TEAEs were mild or moderate in both groups. In patients on TPIP and placebo, TEAEs leading to treatment discontinuation occurred in 4 (13.8%) and 3 (30.0%) patients, respectively, and serious AEs occurred in 6 (20.7%) and 4 (40.0%) patients, respectively. Following the 640\u2009\u00b5g TPIP dose, the treprostinil elimination half\u2010life was 7.1\u2009h. In TPIP and placebo groups, clinical worsening was observed in 3 (10.3%) and 5 (50.0%) patients, respectively. Trends for improvement were observed in 6MWD and NT\u2010proBNP with TPIP treatment. These results suggest that TPIP may be a suitable once\u2010daily treatment for PH\u2010ILD.\n                  <\/jats:p>","DOI":"10.1002\/pul2.70322","type":"journal-article","created":{"date-parts":[[2026,6,15]],"date-time":"2026-06-15T13:28:20Z","timestamp":1781530100000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["A Phase 2 Study of the Safety and Tolerability of Treprostinil Palmitil Inhalation Powder in Patients With Pulmonary Hypertension Associated With Interstitial Lung Disease"],"prefix":"10.1002","volume":"16","author":[{"given":"Colin","family":"Church","sequence":"first","affiliation":[{"name":"Golden Jubilee National University Hospital and University of Glasgow Glasgow UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Molina\u2010Molina","sequence":"additional","affiliation":[{"name":"Interstitial Lung Disease Unit, Respiratory Department, Research Lab 4126, IDIBELL, UB University Hospital of Bellvitge Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miguel","family":"Arias\u2010Guill\u00e9n","sequence":"additional","affiliation":[{"name":"Respiratory Department, Central University Hospital of Asturias University of Oviedo School of Medicine, ISPA; CIBERES (ISCIII) Oviedo Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick","family":"Muchmore","sequence":"additional","affiliation":[{"name":"FLUIDDA, Inc. New York  New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ben R.","family":"Lavon","sequence":"additional","affiliation":[{"name":"FLUIDDA, Inc. New York  New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan De","family":"Backer","sequence":"additional","affiliation":[{"name":"FLUIDDA, Inc. 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Bethesda (MD): National Library of Medicine (US) National Center for Biotechnology Information; 2004\u2010. PubChem Compound Summary for CID 6918140 Treprostinil; [cited2026March 16] https:\/\/pubchem.ncbi.nlm.nih.gov\/compound\/Treprostinil."},{"key":"e_1_2_12_11_1","unstructured":"United Therapeutics Corp. TYVASO\u00ae (treprostinil) Inhalation Solution Prescribing Information; [cited 2026 March 6] https:\/\/www.accessdata.fda.gov\/drugsatfda_docs\/label\/2022\/022387s020lbl.pdf."},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2008470"},{"key":"e_1_2_12_13_1","unstructured":"M. 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