Study of the Efficacy and Safety of Inhaled Treprostinil in Subjects With Progressive Pulmonary Fibrosis (TETON-PPF)
Launched by UNITED THERAPEUTICS · Jul 5, 2023
Trial Information
Current as of July 22, 2025
Recruiting
Keywords
ClinConnect Summary
The TETON-PPF clinical trial is studying a medication called inhaled treprostinil, which is being tested for its safety and effectiveness in treating people with progressive pulmonary fibrosis (PPF), a condition that causes scarring in the lungs and difficulty breathing. The trial will last for 52 weeks and is currently recruiting participants aged 18 and older who have been diagnosed with PPF and have shown signs that their condition is getting worse despite standard treatments.
To be eligible for this trial, participants must give their consent, have specific lung function measurements, and be on certain treatments for their disease. Those who join the study can expect regular check-ins with the research team to monitor their health and response to the medication. It's important to know that this study is looking for individuals who have not had certain complications or treatments recently, and that both men and women of childbearing potential will need to follow specific guidelines to ensure safety during the trial.
Gender
ALL
Eligibility criteria
- 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), or soluble guanylate cyclase stimulators 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.
About United Therapeutics
United Therapeutics is a leading biotechnology company dedicated to addressing the unmet medical needs of patients with life-threatening conditions, particularly in the fields of pulmonary arterial hypertension and organ transplantation. Founded in 1996, the company focuses on the development and commercialization of innovative therapies that enhance patient quality of life and extend survival. With a robust pipeline and a commitment to scientific excellence, United Therapeutics partners with healthcare providers, researchers, and advocacy groups to drive advancements in treatment options and improve outcomes for patients worldwide.
Contacts
Jennifer Cobb
Immunology at National Institute of Allergy and Infectious Diseases (NIAID)
Locations
Rochester, Minnesota, United States
Philadelphia, Pennsylvania, United States
Kansas City, Kansas, United States
Orange, California, United States
Boston, Massachusetts, United States
Durham, North Carolina, United States
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Philadelphia, Pennsylvania, United States
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Greenville, North Carolina, United States
Charlottesville, Virginia, United States
Heidelberg, Victoria, Australia
Kogarah, New South Wales, Australia
Montreal, Quebec, Canada
Taichung, , Taiwan
Baltimore, Maryland, United States
Kaohsiung, , Taiwan
Seoul, , Korea, Republic Of
Petach Tikva, , Israel
Seongnam Si, , Korea, Republic Of
Jerusalem, , Israel
Incheon, , Korea, Republic Of
Liège, , Belgium
Seoul, , Korea, Republic Of
Mckinney, Texas, United States
Royal Oak, Michigan, United States
Tel Aviv, , Israel
Box Hill, Victoria, Australia
Kfar Saba, , Israel
Columbus, Ohio, United States
Chesterfield, Missouri, United States
Greensboro, North Carolina, United States
Los Angeles, California, United States
Clayton, Victoria, Australia
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Bron, , France
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Richmond, Virginia, United States
Salt Lake City, Utah, United States
Newport Beach, California, United States
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Phoenix, Arizona, United States
Kaohsiung City, , Taiwan
Caen, , France
Birmingham, Alabama, United States
Rouen, , France
Tampa, Florida, United States
Knoxville, Tennessee, United States
Franklin, Tennessee, United States
Mcallen, Texas, United States
Chicago, Illinois, United States
Sacramento, California, United States
Houston, Texas, United States
Houston, Texas, United States
North Dartmouth, Massachusetts, United States
Columbia, South Carolina, United States
San Diego, California, United States
Chicago, Illinois, United States
Minneapolis, Minnesota, United States
Ajax, Ontario, Canada
Jacksonville, Florida, United States
Falls Church, Virginia, United States
Québec, Quebec, Canada
New Orleans, Louisiana, United States
Charleston, South Carolina, United States
Milwaukee, Wisconsin, United States
Stony Brook, New York, United States
Lexington, Kentucky, United States
Louisville, Kentucky, United States
Atlanta, Georgia, United States
Toledo, Ohio, United States
Hamilton, Ontario, Canada
Hershey, Pennsylvania, United States
Vancouver, British Columbia, Canada
Brussels, , Belgium
Christchurch, Canterbury, New Zealand
Nashville, Tennessee, United States
Midland, West Australia, Australia
Hadera, , Israel
Ciudad Autonoma De Buenos Aires, , Argentina
Ciudad Autónoma De Buenos Aires, , Argentina
Kfar Saba, , Israel
San Miguel De Tucumán, Tucumán, Argentina
Auckland, , New Zealand
Río Cuarto, Cordoba, Argentina
Cordoba, , Argentina
Haifa, , Israel
San Miguel De Tucuman, Tucuman, Argentina
Santiago, Region Metropolitana, Chile
Quillota, Valparaiso, Chile
Reẖovot, , Israel
Santiago, Region Metropolitana, Chile
Santiago, Region Metropolitana, Chile
Rosario, Santa Fe, Argentina
Curicó, Maule, Chile
Seoul, , Korea, Republic Of
Córdoba, , Argentina
Brisbane, Queensland, Australia
Viña Del Mar, Valparaíso, Chile
Mar Del Plata, Buenos Aires, Argentina
Aalst, , Belgium
Bobigny, , France
Marseille, , France
Tours Cedex 9, , France
Dallas, Texas, United States
Brussels, , Belgium
San Miguel De Tucumán, , Argentina
Viña Del Mar, , Chile
Rosenheim, Bayern, Germany
Redding, California, United States
Löwenstein, , Germany
Coswig, , Germany
München, , Germany
Patients applied
Timeline
First submit
Trial launched
Trial updated
Estimated completion
Not reported