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Search / Trial NCT05943535

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

Treprostinil Ppf Ild

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.

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

Bronx, New York, United States

Saint Louis, Missouri, United States

Maywood, Illinois, United States

Jacksonville, Florida, United States

Dallas, Texas, United States

Stanford, California, United States

Philadelphia, Pennsylvania, United States

Albuquerque, New Mexico, United States

Boston, Massachusetts, United States

Washington, District Of Columbia, United States

Cincinnati, Ohio, United States

Camperdown, New South Wales, Australia

Kansas City, Missouri, United States

Murray, Utah, United States

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

New Hyde Park, New York, United States

Bron, , France

Chicago, Illinois, United States

Nedlands, Western Australia, Australia

Silver Spring, Maryland, United States

Madison, Wisconsin, United States

Richmond, Virginia, United States

Salt Lake City, Utah, United States

Newport Beach, California, United States

New York, New York, United States

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

0 patients applied

Timeline

First submit

Trial launched

Trial updated

Estimated completion

Not reported