Study of the Efficacy and Safety of Inhaled Treprostinil in Subjects With Progressive Pulmonary Fibrosis (TETON-PPF)

May 22, 2026 updated by: United Therapeutics

A Randomized, Double-blind, Placebo-controlled, Multinational, Phase 3 Study of the Efficacy and Safety of Inhaled Treprostinil in Subjects With Progressive Pulmonary Fibrosis (TETON-PPF)

Study RIN-PF-305 is designed to evaluate the safety and efficacy of inhaled treprostinil in subjects with progressive pulmonary fibrosis (PPF) over a 52-week period.

Study Overview

Status

Recruiting

Conditions

Intervention / Treatment

Detailed Description

Study RIN-PF-305 is a Phase 3, multinational, randomized, double-blind, placebo-controlled study to evaluate the safety and efficacy of inhaled treprostinil in subjects with PPF over a 52-week period. Subjects will be randomly allocated 1:1 to receive inhaled treprostinil or placebo. All subjects will initiate inhaled treprostinil or placebo at a dose of 3 breaths administered 4 times daily (QID) and will titrate to a target dosing regimen of 12 breaths QID. Study drug doses may be titrated up as tolerated, until the target dose or maximum clinically tolerated dose is achieved. Once eligible, 6 Treatment Period visits to the clinic will be required at Weeks 4, 8, 16, 28, 40, and 52.

Efficacy assessments include spirometry (forced vital capacity [FVC]), time to clinical worsening, time to first acute exacerbation of interstitial lung disease (ILD), overall survival, King's Brief Interstitial Lung Disease (K-BILD) questionnaire, plasma N-terminal pro-brain natriuretic peptide (NT-proBNP) concentration, supplemental oxygen use, and lung diffusion capacity (DLCO). Safety assessments include the development of adverse events (AEs)/serious adverse events (SAEs), vital signs, clinical laboratory parameters, and electrocardiogram (ECG) parameters.

Subjects who complete the Week 52 Visit may be offered the opportunity to enter an open-label extension (OLE) study after completing the final study visit.

Study Type

Interventional

Enrollment (Estimated)

698

Phase

  • Phase 3

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Contact

Study Locations

  • Argentina
      • Buenos Aires, Argentina, C1426ABP
        • Recruiting
        • Fundacion Respirar
        • Contact:
        • Principal Investigator:
          • María Cristina De Salvo, MD
      • Buenos Aires, Argentina, C1056ABI
        • Recruiting
        • CINME Centro de Investigaciones Metabolicas
        • Contact:
        • Principal Investigator:
          • Maria Otaola, MD
      • Córdoba, Argentina, 5021
        • Recruiting
        • Sanatorio Allende Cerro
        • Contact:
        • Principal Investigator:
          • Viviana Alejandra Moyano
      • Córdoba, Argentina, 5003
        • Recruiting
        • Instituto de Medicina Respiratoria
        • Principal Investigator:
          • Alexis Cazaux, MD
        • Contact:
    • Buenos Aires
      • Mar del Plata, Buenos Aires, Argentina, 7600
        • Recruiting
        • Instituto Ave Pulmo - Fundacion Enfisema
        • Contact:
        • Principal Investigator:
          • Luis Arturo Wehbe, MD
    • Córdoba Province
      • Río Cuarto, Córdoba Province, Argentina, 5800
        • Recruiting
        • Instituto Medico Rio Cuarto
        • Principal Investigator:
          • Mauricio J Eceiza, MD
        • Contact:
    • Santa Fe Province
      • Rosario, Santa Fe Province, Argentina, 2000
        • Recruiting
        • Sanatorio Parque de Rosario - Consultorios Externos
        • Contact:
        • Principal Investigator:
          • Silvina Mannarino, MD
    • Tucumán Province
      • San Miguel de Tucumán, Tucumán Province, Argentina, 4000
        • Recruiting
        • Investigaciones en Patologias Respiratorias
        • Contact:
        • Principal Investigator:
          • Ramon A Rojas, MD
      • San Miguel de Tucumán, Tucumán Province, Argentina
        • Recruiting
        • CIMER-Centro Integral de Medicina Respiratoria
        • Contact:
        • Principal Investigator:
          • Altieri Hector, MD
  • Australia
    • New South Wales
      • Camperdown, New South Wales, Australia, 2050
        • Recruiting
        • Royal Prince Alfred Hospital
        • Contact:
        • Principal Investigator:
          • Tamera Corte, Prof.
      • Kogarah, New South Wales, Australia, 2217
    • Queensland
      • Brisbane, Queensland, Australia, Australia
        • Recruiting
        • The Prince Charles Hospital
        • Contact:
        • Principal Investigator:
          • John Andrew Mackintosh
    • Victoria
      • Box Hill, Victoria, Australia, 3128
        • Recruiting
        • Eastern Health Box Hill Hospital
        • Contact:
        • Principal Investigator:
          • Francis Thien, MD
      • Clayton, Victoria, Australia, 3168
        • Recruiting
        • Monash Health-Monash Medical Centre
        • Principal Investigator:
          • Xun Li, MD
        • Contact:
      • Heidelberg, Victoria, Australia, 3084
        • Recruiting
        • Austin Health
        • Contact:
        • Principal Investigator:
          • Nicole Soo Goh, MD
    • Western Australia
      • Midland, Western Australia, Australia, 6056
        • Recruiting
        • Institute for Respiratory Health - Midland
        • Contact:
        • Principal Investigator:
          • David Manners, MD
      • Nedlands, Western Australia, Australia, 6009
        • Recruiting
        • Institute for Respiratory Health
        • Contact:
        • Principal Investigator:
          • Vidya Navaratnam, MD
  • Belgium
      • Aalst, Belgium, 9300
        • Recruiting
        • AZORG
        • Principal Investigator:
          • Valerie Adam, MD
        • Contact:
      • Antwerp, Belgium, 2020
        • Recruiting
        • ZAS Middelheim
        • Contact:
        • Principal Investigator:
          • Hans Slabbynck, MD
      • Brussels, Belgium, 1200
        • Recruiting
        • Cliniques Universitaires St.-Luc
        • Contact:
        • Principal Investigator:
          • Antoine Froidure, MD
      • Brussels, Belgium, 1070
      • Leuven, Belgium, 3000
        • Recruiting
        • UZ Leuven
        • Contact:
        • Principal Investigator:
          • Wim Wuyts, MD
      • Liège, Belgium, 4000
        • Recruiting
        • CHU de Liège
        • Principal Investigator:
          • Julien Guiot, MD
        • Contact:
  • Canada
    • British Columbia
      • Vancouver, British Columbia, Canada, V6Z 176
        • Recruiting
        • St.Paul's Hospital
        • Principal Investigator:
          • Christopher Ryerson, MD
        • Contact:
    • Ontario
      • Ajax, Ontario, Canada, L1S 2J5
        • Recruiting
        • Dynamic Drug Advancement Limited
        • Principal Investigator:
          • George Philteos, MD
        • Contact:
      • Hamilton, Ontario, Canada, L8N 4A6
        • Recruiting
        • St.Joseph's Healthcare Hamilton
        • Principal Investigator:
          • Nathan Hambly, MD
        • Contact:
    • Quebec
      • Montreal, Quebec, Canada, H1T 2M4
        • Recruiting
        • Hôpital Maisonneuve-Rosemont
        • Contact:
        • Principal Investigator:
          • Ping Shi Zhu, MD
      • Québec, Quebec, Canada, G8T 7A1
        • Recruiting
        • CIC Mauricie inc.
        • Contact:
        • Principal Investigator:
          • Émilie Millaire, MD
      • Sherbrooke, Quebec, Canada, J1E 0N8
        • Recruiting
        • Diex Recherche Inc. - Division Sherbrooke Est
        • Contact:
        • Principal Investigator:
          • Christophe He, MD
  • Chile
    • Maule Region
      • Curicó, Maule Region, Chile, 3341643
        • Recruiting
        • Centro de Investigacion Curico
        • Contact:
        • Principal Investigator:
          • Victor Antonio Martinez Hagen, MD
    • Santiago Metropolitan
      • Santiago, Santiago Metropolitan, Chile, 7550495
        • Recruiting
        • CEC Centro Estudios Clinicos
        • Principal Investigator:
          • Georgina Viviana Miranda Saez, MD
        • Contact:
      • Santiago, Santiago Metropolitan, Chile, 7630226
        • Recruiting
        • Fundacion Medica San Cristobal
        • Contact:
        • Principal Investigator:
          • Ricardo Luis Sepulveda Moncayo, MD
      • Santiago, Santiago Metropolitan, Chile, 8320000
        • Recruiting
        • Biocinetic Ltda.
        • Contact:
        • Principal Investigator:
          • Ricardo Alexis Fritz Garrido, MD
    • Valparaiso
      • Quillota, Valparaiso, Chile, 2260000
        • Recruiting
        • Centro Respiratorio Integral LTDA. (CENRESIN)
        • Contact:
        • Principal Investigator:
          • Juana Rosa Pavie Gallegos, MD
      • Viña del Mar, Valparaiso, Chile, 2520000
        • Recruiting
        • Centro de Investigación de Enfermedades Respiratorias e Inmunológicas (CIERI)
        • Contact:
        • Principal Investigator:
          • Felipe Eduardo Saavedra Gonzalez, MD
  • France
      • Bobigny, France, 93000
        • Recruiting
        • Hôpital Avicennes
        • Contact:
        • Principal Investigator:
          • Hilario Nunes, MD
      • Bron, France, 69500
        • Recruiting
        • Hospices Civils de Lyon - Hopital Louis Pradel
        • Principal Investigator:
          • Vincent Cottin, MD
        • Contact:
      • Caen, France, 14000
        • Recruiting
        • CHU Caen Normandie
        • Principal Investigator:
          • Emmanuel Bergot, MD
        • Contact:
      • Marseille, France
        • Recruiting
        • Assistance Publique Hôpitaux de Marseille - centre Hospitalier Régional de Marseille
        • Principal Investigator:
          • Martine Reynaud-Gaubert, MD
        • Contact:
      • Rouen, France, 7600
        • Recruiting
        • Rouen University Hospital
        • Principal Investigator:
          • Mathieu Salaun, MD
        • Contact:
      • Tours, France, Tours Cedex 9
        • Recruiting
        • Centre Hospitalier Régional Universitaire de Tours
        • Principal Investigator:
          • Julie Mankikian, MD
        • Contact:
  • Germany
      • Bad Berka, Germany, 99437
        • Recruiting
        • Zentralklinik Bad Berka GmbH Klinik für Pneumologie, Zentrum für Schlaf-und Beatmungsmedizin
        • Principal Investigator:
          • Michael Weber, MD
        • Contact:
      • Coswig, Germany, 01640
        • Recruiting
        • Prufstelle der GWT am Fachkrankenhaus Coswig
        • Contact:
        • Principal Investigator:
          • Dirk Koschel, MD
      • Essen, Germany, 45239
        • Recruiting
        • uhrlandklinik Westdeutsches Lungenzentrum am Universitätsklinikum Essen gGmbH
        • Contact:
        • Principal Investigator:
          • Francesco T Bonella, MD
      • Löwenstein, Germany, 74245
        • Recruiting
        • SLK Kliniken Heilbronn GmbH
        • Contact:
        • Principal Investigator:
          • Jonas Kuon, Dr
      • München, Germany, 81377
        • Recruiting
        • LMU Klinikum Medizinische Klinik und Poliklinik V
        • Contact:
        • Principal Investigator:
          • Jurgen Behr, MD
    • Bavaria
      • Rosenheim, Bavaria, Germany, 83022
        • Recruiting
        • RoMed Klinikum Rosenheim-Ellmaierstr. 23
        • Contact:
        • Principal Investigator:
          • Stephan Budweiser, MD
  • Israel
      • Ashkelon, Israel, 7830604
        • Recruiting
        • Barzilai Medical Center
        • Contact:
        • Principal Investigator:
          • Ori Wand, MD
      • Hadera, Israel, 3846201
        • Recruiting
        • Hillel Yaffe Medical Center
        • Contact:
        • Principal Investigator:
          • Michael Kuchuk, MD
      • Haifa, Israel, 3436212
        • Recruiting
        • Rabin Medical Center
        • Contact:
        • Principal Investigator:
          • Yochai Adir, Prof
      • Jerusalem, Israel, 9103102
        • Recruiting
        • Shaare Zedek Medical Center
        • Contact:
        • Principal Investigator:
          • Gabriel Izbicki, MD
      • Jerusalem, Israel, 9112001
        • Recruiting
        • The Hadassah University Medical Center Ein Kerem Hospital
        • Principal Investigator:
          • Neville Berkman, Prof.
        • Contact:
      • Kfar Saba, Israel, 4428164
        • Recruiting
        • Meir Medical Center
        • Principal Investigator:
          • David Shitrit, MD
        • Contact:
      • Petah Tikva, Israel, 4941492
        • Recruiting
        • Rabin Medical Center
        • Principal Investigator:
          • Mordechai Kramer, MD
        • Contact:
      • Rehovot, Israel, 7551041
        • Recruiting
        • Kaplan Medical Center
        • Contact:
        • Principal Investigator:
          • Alexander Yarmolovsky, MD
      • Tel Aviv, Israel, 6423906
        • Recruiting
        • Tel Aviv Sourasky Medical Center
        • Principal Investigator:
          • Amir Bar-Shai, MD
        • Contact:
  • Italy
      • Catania, Italy, 95123
        • Recruiting
        • Azienda Ospedaliero Universitaria Policlinico "G. Rodolico - San Marco" Presidio Ospedaliero Gaspare Rodolico
        • Principal Investigator:
          • Carlo Vancheri
        • Contact:
      • Forlì, Italy, 47121
        • Recruiting
        • Azienda Unita Sanitaria Locale Della Romagna Ospedale "Gian Battista Morgagni - Luigi Pierantoni" di Forlì
        • Principal Investigator:
          • Claudia Ravaglia, MD
        • Contact:
      • Modena, Italy, 41124
        • Recruiting
        • Azienda Ospedaliero-Universitaria di Modena Policlinico di Modena
        • Principal Investigator:
          • Stefania Cerri, MD
        • Contact:
      • Roma, Italy, 168
        • Recruiting
        • Fondazione Policlinico Universitario Agostino Gemelli IRCCS
        • Principal Investigator:
          • Luca Richeldi
        • Contact:
      • Siena, Italy, 53100
        • Recruiting
        • Azienda Ospedaliero-Universitaria Senese
        • Principal Investigator:
          • Elena Bargagli, MD
        • Contact:
  • New Zealand
      • Auckland, New Zealand
        • Recruiting
        • Aotearoa Clinical Trials Trust- Middlemore Hospital
        • Principal Investigator:
          • Conor Odochartaigh, MD
        • Contact:
      • Hamilton, New Zealand, 3204
        • Recruiting
        • Waikato Hospital
        • Principal Investigator:
          • Catherina Chang
        • Contact:
    • Canterbury
      • Christchurch, Canterbury, New Zealand, 8011
        • Recruiting
        • Canterbury Respiratory Research Group
        • Principal Investigator:
          • Michael Epton, MD
        • Contact:
  • Peru
      • Cusco, Peru, 08002
        • Recruiting
        • Hospital Nacional Adolfo Guevara Velasco
        • Contact:
        • Principal Investigator:
          • Sandra Graciela Vargas Marquez, MD
      • Lima, Peru, 15036
        • Recruiting
        • Clinica Ricardo Palma
        • Contact:
        • Principal Investigator:
          • Cesar Fernando Villaran Ferreyros, MD
      • Lima, Peru, 15046
        • Recruiting
        • Hospital Central de la Fuerza Aerea del Peru
        • Contact:
        • Principal Investigator:
          • Maria Carmela Faverio Mannucci, MD
      • Lima, Peru, 15023
        • Recruiting
        • Unidad de Investigacion de Enfermedades Respiratorias- Clinica San Pablo -Surco
        • Contact:
        • Principal Investigator:
          • Alejandro M Peña Villalobos, MD
      • Lima, Peru, 15088
        • Recruiting
        • Centro de Investigacion de Enfermedades Respiratorias - Clinica Providencia
        • Contact:
        • Principal Investigator:
          • Carlos A Iberico Barrera, MD
      • Lima, Peru, 15001
        • Recruiting
        • Clinica Internacional - Sede Lima
        • Contact:
        • Principal Investigator:
          • Alfredo Guerreros Benavides, MD
  • South Korea
      • Incheon, South Korea, 21565
        • Recruiting
        • Gachon University Gil Medical Center
        • Principal Investigator:
          • Sung Hwan Jeong, MD
        • Contact:
      • Seongnam-si, South Korea, 13620
        • Recruiting
        • Seoul National University Bundang Hospital
        • Principal Investigator:
          • Jong Sun Park, MD
        • Contact:
      • Seoul, South Korea, 05505
        • Recruiting
        • Asan Medical Center
        • Principal Investigator:
          • Jin Woo Song, MD
        • Contact:
      • Seoul, South Korea, 02841
        • Recruiting
        • Korea University Anam Hospital
        • Principal Investigator:
          • Eun Joo Lee, MD
        • Contact:
      • Seoul, South Korea, 3312
        • Recruiting
        • The Catholic University of Korea, Eunpyeong St. Mary's Hospital
        • Principal Investigator:
          • Sang Haak Lee, MD
        • Contact:
  • Taiwan
      • Kaohsiung City, Taiwan, 807
        • Recruiting
        • Kaohsiung Medical University Chung-Ho Memorial Hospital
        • Contact:
        • Principal Investigator:
          • Sheu Chau-Chyun, MD
      • Kaohsiung City, Taiwan, 83301
        • Recruiting
        • Chang Gung Medical Foundation Kaohsiung Chang Gung Memorial Hospital
        • Contact:
        • Principal Investigator:
          • Kuo-Tung Huang, MD
      • Taichung, Taiwan, 40705
        • Recruiting
        • Taichung Veterans General Hospital
        • Contact:
        • Principal Investigator:
          • Pin-Kuei Fu, MD
  • United Kingdom
      • Birmingham, United Kingdom
        • Recruiting
        • Birmingham Heartlands Hospital
        • Contact:
        • Principal Investigator:
          • Christopher Huntley, MD
      • Cambridge, United Kingdom, CB2 0AY
        • Recruiting
        • Royal Papworth Hospital
        • Contact:
        • Principal Investigator:
          • Christine Fiddley, MD
      • Edinburgh, United Kingdom, EH16 4SA
        • Recruiting
        • NHS Lothian, Royal INfirmary of Edingburgh
        • Principal Investigator:
          • Nikhil Hirani, MD
        • Contact:
      • London, United Kingdom, SE1 9RT
        • Recruiting
        • Guy'S Hospital
        • Principal Investigator:
          • Katherine Myall, MD
        • Contact:
      • Manchester, United Kingdom, M8 5RB
        • Recruiting
        • Manchester University NHS Foundation Trust, North Manchester General Hospital
        • Contact:
        • Principal Investigator:
          • Zoe Borril, MD
      • Perth, United Kingdom, PH1 1NX
        • Recruiting
        • NHS Tayside, Perth Royal Infirmary
        • Principal Investigator:
          • Mark Spears, MD
        • Contact:
  • United States
    • Alabama
      • Birmingham, Alabama, United States, 35233
        • Recruiting
        • UAB Lung Health Center
        • Contact:
        • Principal Investigator:
          • Tejaswini Kulkarni, MD
    • Arizona
      • Phoenix, Arizona, United States, 85013
        • Recruiting
        • Norton Thoracic Institute
        • Contact:
        • Principal Investigator:
          • Rajat Walia, MD
      • Phoenix, Arizona, United States, 85006
        • Recruiting
        • Banner University Medical Center Phoenix Lung Institute
        • Principal Investigator:
          • Sally Suliman, MD
        • Contact:
    • California
      • Los Angeles, California, United States, 90095
        • Recruiting
        • Peter Morton Medical Building
        • Contact:
        • Principal Investigator:
          • Stephen S Weigt, MD
      • Newport Beach, California, United States, 92663
        • Recruiting
        • NewportNativeMD, Inc.
        • Principal Investigator:
          • Ryan Klein, MD
        • Contact:
      • Orange, California, United States, 92868
        • Recruiting
        • University of California Irvine Medical Center
        • Principal Investigator:
          • Bassam Yaghmour, MD
        • Contact:
      • Redding, California, United States, 45227
        • Recruiting
        • Paradigm Clinical Research
        • Principal Investigator:
          • Rafael Lupercio, MD
        • Contact:
      • Sacramento, California, United States, 95817
        • Recruiting
        • UC Davis Health Medical Center
        • Principal Investigator:
          • Namita Sood, MD
        • Contact:
      • San Diego, California, United States, 92108
        • Recruiting
        • Paradigm Clinical Research
        • Contact:
        • Principal Investigator:
          • Daniel Jones, MD
      • Stanford, California, United States, 94305
        • Recruiting
        • Stanford University Medical Center
        • Principal Investigator:
          • Rishi Raj, MD
        • Contact:
    • District of Columbia
      • Washington D.C., District of Columbia, United States, 20007
        • Recruiting
        • Georgetown University Hospital
        • Contact:
        • Principal Investigator:
          • Cristina Reichner, MD
    • Florida
      • Jacksonville, Florida, United States, 32224
        • Recruiting
        • Mayo Clinic
        • Contact:
        • Principal Investigator:
          • Augustine S Lee, MD
      • Jacksonville, Florida, United States, 32204
        • Recruiting
        • Ascension Medical Group St. Vincent's Lung Institute
        • Contact:
        • Principal Investigator:
          • Akram Shibani, MD
      • Tampa, Florida, United States, 33606
        • Recruiting
        • TGH/USF Center for Advanced Lung Disease and Lung Transplant
        • Contact:
        • Principal Investigator:
          • Debabrata Bandyopadhyay, MD
    • Georgia
      • Atlanta, Georgia, United States, 45227
        • Recruiting
        • The Emory Clinic
        • Contact:
        • Principal Investigator:
          • Srihari Veeraraghavan, MD
    • Illinois
      • Chicago, Illinois, United States, 60612
        • Recruiting
        • Rush University Medical Center Outpatient Pulmonary Clinic
        • Contact:
        • Principal Investigator:
          • Mark Yoder, MD
      • Chicago, Illinois, United States, 60612
        • Recruiting
        • UI Health Hospital
        • Principal Investigator:
          • Dustin Fraidenburg, MD
        • Contact:
      • Chicago, Illinois, United States, 60611
        • Recruiting
        • Northwestern Memorial Hospital, Clinical Research Unit
        • Contact:
        • Principal Investigator:
          • Bradford Bemiss, MD
      • Maywood, Illinois, United States, 60153
        • Recruiting
        • Loyola University Medical Center
        • Principal Investigator:
          • Daniel Dilling, MD
        • Contact:
    • Kansas
      • Kansas City, Kansas, United States, 66160
        • Recruiting
        • University of Kansas Medical Center
        • Contact:
        • Principal Investigator:
          • Sahil Pandya, MD
    • Kentucky
      • Lexington, Kentucky, United States, 45227
        • Recruiting
        • University Of Kentucky
        • Principal Investigator:
          • Parijat Sen, MD
        • Contact:
          • Srushan Shankara bhaktula
          • Phone Number: 859-562-0882
          • Email: ssh486@uky.edu
      • Louisville, Kentucky, United States, 40202
        • Recruiting
        • University of Louisville Healthcare Outpatient Research Clinic
        • Contact:
        • Principal Investigator:
          • Mohamed A Saad, MD
    • Louisiana
      • New Orleans, Louisiana, United States, 45227
        • Recruiting
        • Tulane Medical Center
        • Contact:
        • Principal Investigator:
          • Joseph Lasky, MD
    • Maryland
      • Baltimore, Maryland, United States, 21224
        • Recruiting
        • Johns Hopkins Asthma and Allergy Center
        • Contact:
        • Principal Investigator:
          • Mathew Lammi, MD
      • Baltimore, Maryland, United States, 45227
        • Recruiting
        • University of Maryland Medical Center
        • Contact:
        • Principal Investigator:
          • Sarah R Williams
      • Silver Spring, Maryland, United States, 20904
        • Recruiting
        • Adventist Healthcare White Oak Medical Center
        • Principal Investigator:
          • Jonathan Cohen, MD
        • Contact:
    • Massachusetts
      • Boston, Massachusetts, United States, 02114
        • Recruiting
        • Massachusetts General Hospital
        • Contact:
        • Principal Investigator:
          • Leo C Ginns, MD
      • Boston, Massachusetts, United States, 02111
        • Recruiting
        • Tufts Medical Center
        • Principal Investigator:
          • Nicholas Hill, MD
        • Contact:
      • North Dartmouth, Massachusetts, United States, 02747
        • Recruiting
        • Infinity Medical Center
        • Contact:
        • Principal Investigator:
          • Curtis J Mello, MD
    • Michigan
      • Grand Rapids, Michigan, United States, 49503
        • Recruiting
        • Corewell Health Grand Rapids Hospitals - Butterworth
        • Contact:
        • Principal Investigator:
          • Pranav Kidambi, MD
      • Royal Oak, Michigan, United States, 48073
        • Recruiting
        • Beaumont Hospital, Royal Oak
        • Contact:
        • Principal Investigator:
          • Enrique Calvo-Ayala, MD
    • Minnesota
      • Minneapolis, Minnesota, United States, 55455
        • Recruiting
        • University of Minnesota Health Clinical Research Unit (CRU)
        • Principal Investigator:
          • Hyun Kim, MD
        • Contact:
      • Rochester, Minnesota, United States, 55905
        • Recruiting
        • Mayo Clinic
        • Principal Investigator:
          • Teng Moua, MD
        • Contact:
    • Mississippi
      • Gulfport, Mississippi, United States, 39501
        • Recruiting
        • Memorial Hospital at Gulfport
        • Principal Investigator:
          • Bobby W Tullos, MD
    • Missouri
      • Chesterfield, Missouri, United States, 63017
        • Recruiting
        • The Lung Research Center, LLC
        • Contact:
        • Principal Investigator:
          • Neil Ettinger, MD
      • Kansas City, Missouri, United States, 64111
        • Recruiting
        • Saint Luke's Hospital of Kansas City
        • Principal Investigator:
          • Mark Yagan, MD
        • Contact:
      • St Louis, Missouri, United States, 63110
        • Recruiting
        • Washington University School of Medicine
        • Contact:
        • Principal Investigator:
          • Tonya D. Russell, MD
    • New Mexico
      • Albuquerque, New Mexico, United States, 87131
        • Recruiting
        • University of New Mexico
        • Contact:
        • Principal Investigator:
          • Nour Assad, MD
    • New York
      • New Hyde Park, New York, United States, 11042
        • Recruiting
        • Northwell Health
        • Principal Investigator:
          • Arunabh Talwar, MD
        • Contact:
      • New York, New York, United States, 10065
        • Recruiting
        • Weill Cornell Medicine, New York-Presbyterian Hospital
        • Contact:
        • Principal Investigator:
          • Anna Podolanczuk, MD
      • Stony Brook, New York, United States, 11794-8172
      • The Bronx, New York, United States, 10467
        • Recruiting
        • Montefiore Medical Center
        • Contact:
        • Principal Investigator:
          • Ali Mansour, MD
    • North Carolina
      • Durham, North Carolina, United States, 27710
        • Recruiting
        • Duke University Medical Center
        • Contact:
        • Principal Investigator:
          • Lake Morrison, MD
      • Greensboro, North Carolina, United States, 27403
        • Recruiting
        • Pulmonix LLC
        • Principal Investigator:
          • Murali Ramaswamy, MD
        • Contact:
      • Greenville, North Carolina, United States, 27834
        • Recruiting
        • East Carolina University
        • Contact:
        • Principal Investigator:
          • Veeranna Maddipati, MD
      • Winston-Salem, North Carolina, United States, 27104
        • Recruiting
        • Wake Forest Baptist Health
        • Contact:
        • Principal Investigator:
          • Heather Clark
    • Ohio
      • Cincinnati, Ohio, United States, 45219
        • Recruiting
        • University of Cincinnati Medical Center
        • Principal Investigator:
          • Nishant Gupta, MD
        • Contact:
      • Columbus, Ohio, United States, 43201
        • Recruiting
        • The Ohio State University Wexner Medical Center
        • Contact:
        • Principal Investigator:
          • Nitin Bhatt, MD
      • Toledo, Ohio, United States, 43608
        • Recruiting
        • Mercy Health St.Vincent Medical Center LLC
        • Contact:
        • Principal Investigator:
          • Vinod Khatri, MD
    • Pennsylvania
      • Hershey, Pennsylvania, United States, 17033
        • Recruiting
        • Pennsylvania State Hershey Medical Center and College of Medicine
        • Principal Investigator:
          • Rebecca Bascom, MD
        • Contact:
      • Philadelphia, Pennsylvania, United States, 19140
        • Recruiting
        • Temple University Hospital
        • Contact:
        • Principal Investigator:
          • Rali Parth, MD
      • Philadelphia, Pennsylvania, United States, 19104
        • Recruiting
        • Hospital of the University of Pennsylvania
        • Contact:
        • Principal Investigator:
          • Michaela Anderson, MD
    • South Carolina
      • Charleston, South Carolina, United States, 29425
        • Recruiting
        • Medical University of South Carolina-Nexus
        • Principal Investigator:
          • Rachana Krishna, MD
        • Contact:
      • Columbia, South Carolina, United States, 29203
        • Recruiting
        • Prisma Health Pulmonology-Richland
        • Contact:
        • Principal Investigator:
          • Abhinav Gupta, MD
    • Tennessee
      • Franklin, Tennessee, United States, 37067
        • Recruiting
        • Clinical Trials Center of Middle Tennessee, LLC
        • Principal Investigator:
          • Aaron Milstone, MD
        • Contact:
      • Knoxville, Tennessee, United States, 37919
        • Recruiting
        • StatCare Pulmonary Consultants, PLLC
        • Principal Investigator:
          • Eric Weaver, MD
        • Contact:
      • Nashville, Tennessee, United States, 37204
        • Recruiting
        • The Vanderbilt Lung Institute
        • Principal Investigator:
          • Justin Hewlett, MD
        • Contact:
    • Texas
      • Dallas, Texas, United States, 75246
        • Recruiting
        • Baylor University Medical Center
        • Principal Investigator:
          • Yosafe Wakwaya, MD
        • Contact:
      • Dallas, Texas, United States, 75390
        • Recruiting
        • UT Southwestern Medical Center-Advanced Lung Clinic
        • Contact:
        • Principal Investigator:
          • Margaret Kypreos, MD
      • Houston, Texas, United States, 77030
      • Houston, Texas, United States, 77030
        • Recruiting
        • The University of Texas Health Science Center at Houston, McGovern Medical School
        • Contact:
        • Principal Investigator:
          • Rodeo Abrencillo, MD
      • McAllen, Texas, United States, 78503
        • Recruiting
        • A & A Research Consultants, LLC
        • Contact:
        • Principal Investigator:
          • Alfredo Renzo Arauco Brown, MD
      • McKinney, Texas, United States, 75069
        • Recruiting
        • Metroplex Pulmonary and Sleep Center
        • Contact:
        • Principal Investigator:
          • Shahrukh Kureishy, MD
    • Utah
      • Murray, Utah, United States, 84107
        • Recruiting
        • Intermountain Medical Center
        • Principal Investigator:
          • Peter Crossno, MD
        • Contact:
      • Salt Lake City, Utah, United States, 84108
        • Recruiting
        • University of Utah Health
        • Contact:
        • Principal Investigator:
          • Mary Scholand, MD
    • Virginia
      • Charlottesville, Virginia, United States, 22903
        • Recruiting
        • University of Virginia Health System
        • Contact:
        • Principal Investigator:
          • Hannah C Mannem, MD
      • Falls Church, Virginia, United States, 22042
        • Recruiting
        • Christopher King, MD
        • Contact:
        • Principal Investigator:
          • Christopher King, MD
      • Richmond, Virginia, United States, 23230
        • Recruiting
        • Pulmonary Associates of Richmond, Inc.
        • Principal Investigator:
          • Shilpa Johri, MD
        • Contact:
    • Wisconsin
      • Madison, Wisconsin, United States, 53792
        • Recruiting
        • University Hospital and UW Health Clinics
        • Contact:
        • Principal Investigator:
          • Stephen J Halliday, MD
      • Milwaukee, Wisconsin, United States, 53226-1222
        • Recruiting
        • Medical College of Wisconsin
        • Contact:
        • Principal Investigator:
          • Mark Barash, MD

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

  • Adult
  • Older Adult

Accepts Healthy Volunteers

No

Description

Inclusion Criteria:

  1. Subject gives voluntary informed consent to participate in the study.
  2. Subject is ≥18 years of age, inclusive, at the time of signing informed consent.
  3. Subject has radiological evidence of pulmonary fibrosis of >10% extent on an HRCT scan in the previous 12 months (confirmed by central review).

  4. Subject has a diagnosis of PPF (other than IPF) that fulfills at least 1 of the following criteria for progression within 24 months of screening despite standard treatment of ILD, as assessed by the Investigator:

    1. Clinically significant decline in % predicted FVC based on ≥10% relative decline
    2. Marginal decline in % predicted FVC based on ≥5% to <10% relative decline combined with worsening of respiratory symptoms
    3. Marginal decline in % predicted FVC based on ≥5% to <10% relative decline combined with increasing extent of fibrotic changes on chest imaging
    4. Worsening of respiratory symptoms as well as increasing extent of fibrotic changes on chest imaging
  5. FVC ≥45% predicted at Screening (confirmed by central review).

  6. Subjects must be on 1 of the following:

    1. On nintedanib or pirfenidone for ≥90 days prior to Baseline and in the Investigator's opinion, are planning to continue treatment through the study
    2. Not on treatment with nintedanib or pirfenidone for ≥90 days prior to Baseline and in the Investigator's opinion, not planning to initiate either treatment during the study.

    Concomitant use of both nintedanib and pirfenidone is not permitted.

  7. Subjects treated with immunosuppressive agents (eg, mycophenolate, methotrexate, azathioprine, oral corticosteroids, rituximab) need to be on treatment for at least 120 days prior to Baseline and, in the Investigator's clinical opinion, must be refractory to treatment.

  8. Women of childbearing potential must be non-pregnant (as confirmed by a urine pregnancy test at Screening and Baseline) and non-lactating, and will agree to do 1 of the following:

    1. Abstain from intercourse (when it is in line with their preferred and usual lifestyle)
    2. Use 2 medically acceptable, highly effective forms of contraception for the duration of the study, and at least 30 days after discontinuing study drug.

    i. Medically acceptable, highly effective forms of contraception can include approved hormonal contraceptives (oral, injectable, and implantable) and barrier methods (such as a condom or diaphragm) when used with a spermicide.

    Women who are successfully sterilized (including hysterectomy, bilateral salpingectomy, or bilateral oophorectomy) or postmenopausal (defined as amenorrhea for at least 12 consecutive months) are not considered to be of reproductive potential.

  9. Males with a partner of childbearing potential must agree to use a condom for the duration of treatment and for at least 48 hours after discontinuing study drug.
  10. In the opinion of the Investigator, the subject is able to communicate effectively with study personnel, and is considered reliable, willing, and likely to be cooperative with protocol requirements, including attending all study visits.

Exclusion Criteria:

  1. Subject is pregnant or lactating.
  2. Subject has primary obstructive airway physiology (forced expiratory volume in 1 second/FVC <0.70 at Screening) or greater extent of emphysema than fibrosis on HRCT (confirmed by central review).
  3. Subject has a diagnosis of IPF.
  4. Subject has shown intolerance or significant lack of efficacy to a prostacyclin or prostacyclin analogue that resulted in discontinuation or inability to effectively titrate that therapy.
  5. Subject has received any PAH-approved therapy, including prostacyclin therapy (epoprostenol, treprostinil, iloprost, or beraprost; except for acute vasoreactivity testing), IP receptor agonists (selexipag), endothelin receptor antagonists, phosphodiesterase type 5 inhibitors (PDE5-Is), soluble guanylate cyclase stimulators, or activin signaling inhibitors (sotatercept) within 60 days prior to Baseline. As needed use of a PDE5-I for erectile dysfunction is permitted, provided no doses are taken within 48 hours prior to any study-related efficacy assessments.
  6. Subject is receiving >10 L/min of oxygen supplementation by any mode of delivery at rest at Baseline.
  7. Exacerbation of ILD or active pulmonary or upper respiratory infection within 30 days prior to Baseline. Subjects must have completed any antibiotic or steroid regimens for treatment of the infection or acute exacerbation more than 30 days prior to Baseline to be eligible. If hospitalized for an acute exacerbation of ILD or a pulmonary or upper respiratory infection, subjects must have been discharged more than 90 days prior to Baseline to be eligible.
  8. Subject has uncontrolled cardiac disease, defined as myocardial infarction within 6 months prior to Baseline or unstable angina within 30 days prior to Baseline.
  9. Use of any other investigational drug/device or participation in any investigational study in which the subject received a medical intervention (ie, procedure, device, medication/supplement) within 30 days prior to Screening. Subjects participating in non-interventional, observational, or registry studies are eligible.
  10. Acute pulmonary embolism within 90 days prior to Baseline.
  11. In the opinion of the Investigator, the subject has any condition that would interfere with the interpretation of study assessments or would impair study participation or cooperation.
  12. In the opinion of the Investigator, life expectancy <12 months due to ILD or a concomitant illness.
  13. Subject has received nerandomilast within 60 days prior to Baseline.

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

  • Primary Purpose: Treatment
  • Allocation: Randomized
  • Interventional Model: Parallel Assignment
  • Masking: Quadruple

Arms and Interventions

Participant Group / Arm
Intervention / Treatment
Placebo Comparator: Placebo
Matching placebo inhaled using an ultrasonic nebulizer QID
Drug: Placebo
Placebo administered QID
Device: Treprostinil Ultrasonic Nebulizer
Treprostinil ultrasonic nebulizer which emits a dose of approximately 6 mcg per breath.
Experimental: Inhaled Treprostinil
Treprostinil for inhalation solution (0.6 mg/mL) delivered via an ultrasonic nebulizer which emits a dose of approximately 6 mcg per breath. Inhaled QID and titrated up to a target of 12 breaths QID or until the subject reaches their maximum clinically tolerated dose.
Device: Treprostinil Ultrasonic Nebulizer
Treprostinil ultrasonic nebulizer which emits a dose of approximately 6 mcg per breath.
Drug: Inhaled Treprostinil
Inhaled treprostinil (6 mcg/breath) administered QID
Other Names:
  • Tyvaso

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Measure Description
Time Frame
Change in Absolute FVC from Baseline to Week 52
Time Frame: Baseline to Week 52
The FVC measurement indicates the amount of air a person can forcefully and quickly exhale after taking a deep breath.
Baseline to Week 52

Secondary Outcome Measures

Outcome Measure
Measure Description
Time Frame
Overall Survival at Week 52
Time Frame: Week 52
Vital status will be assessed for all subjects at Week 52, including those who discontinue the study prematurely or who withdraw consent.
Week 52
Time to First Clinical Worsening
Time Frame: Baseline to Week 52
Clinical worsening is monitored from randomization until 1 of the following criteria are met: death (all causes), hospitalization due to a respiratory indication, or ≥10% relative decline in % predicted FVC.
Baseline to Week 52
Time to First Acute Exacerbation of ILD
Time Frame: Baseline to Week 52
An exacerbation of ILD is defined as an acute, clinically significant, respiratory deterioration characterized by evidence of new widespread alveolar abnormality.
Baseline to Week 52
Change in % Predicted FVC from Baseline to Week 52
Time Frame: Baseline to Week 52
The FVC measurement indicates the amount of air a person can forcefully and quickly exhale after taking a deep breath. Percent predicted FVC is calculated based on factors such as race, sex, age, height, and weight.
Baseline to Week 52
Change in K-BILD Questionnaire Score from Baseline to Week 52
Time Frame: Baseline to Week 52
The K-BILD is a self-administered, 15-item questionnaire validated for patients with ILD consisting of 3 domains (breathlessness and activities, psychological, and chest symptoms).
Baseline to Week 52
Change in DLCO from Baseline to Week 52
Time Frame: Baseline to Week 52
The DLCO measurement measures how well oxygen moves from the lungs to the blood.
Baseline to Week 52

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

United Therapeutics

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

October 30, 2023

Primary Completion (Estimated)

November 1, 2027

Study Completion (Estimated)

November 1, 2027

Study Registration Dates

First Submitted

July 5, 2023

First Submitted That Met QC Criteria

July 5, 2023

First Posted (Actual)

July 13, 2023

Study Record Updates

Last Update Posted (Actual)

May 26, 2026

Last Update Submitted That Met QC Criteria

May 22, 2026

Last Verified

May 1, 2026

More Information

Terms related to this study

Keywords

Additional Relevant MeSH Terms

Other Study ID Numbers

  • RIN-PF-305

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

Yes

Studies a U.S. FDA-regulated device product

Yes

product manufactured in and exported from the U.S.

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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