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Looking for participantsPhase3

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

Sponsor: United Therapeutics

NCT ID: NCT05943535

View on ClinicalTrials.gov ↗

At a glance

What the study gets you
Access to the study treatment being tested
Type of study
Interventional (receives a drug or procedure)
Time in hospital
In-person visits at study sites — visit count not specified by the sponsor
Drug or intervention
Placebo (drug), Inhaled Treprostinil (drug), Treprostinil Ultrasonic Nebulizer (device)
How long the study runs
Study runs about 49 months (dates as stated)
About the drug or intervention
Placebo — drug: Placebo administered QID · Inhaled Treprostinil — drug: Inhaled treprostinil (6 mcg/breath) administered QID · Treprostinil Ultrasonic Nebulizer — device: Treprostinil ultrasonic nebulizer which emits a dose of approximately 6 mcg per breath.
Patient visit burden
Not specified by the sponsor

In plain English

This study, run by United Therapeutics, is looking at whether an inhaled medicine called treprostinil is safe and helps people with progressive pulmonary fibrosis (a lung condition where scarring gets worse over time) that is not idiopathic pulmonary fibrosis. Progressive pulmonary fibrosis is a form of interstitial lung disease, a group of conditions causing inflammation and scarring in the lungs.

Who can take part

  • Adults aged 18 or over who agree to take part
  • Have scarring (fibrosis) on a lung scan (HRCT) in the past 12 months, covering more than 10% of the lung, checked by a central review team
  • Have progressive pulmonary fibrosis that is not idiopathic pulmonary fibrosis (IPF), with signs of the disease getting worse in the past 2 years despite standard treatment. Worsening can include: a sizeable drop in breathing test results (10% or more), a smaller drop (5–10%) alongside worse breathlessness or more scarring on scans, or worse symptoms together with more scarring on scans
  • Breathing test (FVC) result of at least 45% of the predicted value at screening, checked by central review
  • Either taking nintedanib or pirfenidone for at least 90 days before the study starts and planning to carry on, or not taking either for at least 90 days and not planning to start. Taking both together is not allowed
  • If taking medicines that dampen the immune system (such as mycophenolate, methotrexate, azathioprine, steroid tablets, or rituximab), must have been on them for at least 120 days and the treatment must not be working well, in the doctor's opinion
  • Women who could become pregnant must not be pregnant or breastfeeding, and must either avoid sex or use two highly effective forms of contraception during the study and for 30 days after stopping the study medicine
  • Men with a partner who could become pregnant must use a condom during treatment and for 48 hours after stopping
  • Able to communicate with the study team and likely to attend all study visits

Who may not be able to

  • Pregnant or breastfeeding
  • Main problem is blocked airways rather than scarring (breathing test ratio below 0.70), or more emphysema than scarring on the lung scan
  • A diagnosis of idiopathic pulmonary fibrosis (IPF)
  • Previous bad reaction or no benefit from prostacyclin or similar medicines, leading to stopping or being unable to adjust the dose
  • Taken any pulmonary hypertension (PAH) medicines — including prostacyclin-type medicines, selexipag, endothelin receptor antagonists, PDE5 inhibitors, or similar — in the 60 days before the study starts (PDE5 inhibitors for erectile dysfunction are allowed, but not within 48 hours before study tests)
  • Needing more than 10 litres per minute of oxygen at rest
  • A flare-up of lung disease or a chest or airway infection in the 30 days before the study starts (or hospital discharge for these within the past 90 days)
  • Uncontrolled heart disease, such as a heart attack in the past 6 months or unstable angina in the past 30 days
  • Taken part in another study with an experimental drug or device in the past 30 days (taking part in observational or registry studies is fine)
  • A blood clot in the lungs (pulmonary embolism) in the past 90 days
  • Any condition that, in the doctor's opinion, would interfere with study tests or taking part
  • Life expectancy of less than 12 months, in the doctor's opinion
  • Taken nerandomilast in the past 60 days

What taking part involves

  • • An inhaled medicine called treprostinil, breathed in through a nebuliser (a device that turns medicine into a fine mist)
  • • Not stated whether everyone gets the active medicine or some get a placebo (dummy) — ask the trial team
  • • You keep taking your usual lung fibrosis medicine (nintedanib or pirfenidone) if you were already on it, but not both together

Time commitment: Not stated — ask the trial team about how many visits are needed, how long the study lasts, and what taking part involves.

Plain-English summary (AI-generated) from registry data. Not eligibility advice — only the trial team can confirm whether you can take part. Use the eligibility checker to see how your health profile matches this trial.

Type of study
Testing a treatment
Ages
18 Years and over
Who
All
Number of participants
698
Started
2023-10-30
Last checked
2026-07

Plain English Summary

What is this study?

  • • Testing a new treatment for progressive pulmonary fibrosis
  • • Phase3 - 698 participants
  • • 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

Who can take part?

  • • Ages 18 Years and over
  • • Diagnosed with progressive pulmonary fibrosis

Where?

  • • Birmingham - Birmingham Heartlands Hospital
  • • Cambridge - Royal Papworth Hospital
  • • Edinburgh - NHS Lothian, Royal INfirmary of Edingburgh
  • • London - Guy's Hospital
  • • +2 more UK sites

This is a simplified summary. Always discuss with your doctor before making any decisions.

About This Trial

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.

More detail

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.

Progressive Pulmonary FibrosisInterstitial Lung Disease

How this trial compares with your answers

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What we know so far

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Preliminary match based on your answers. Full eligibility requires on-site assessment including medical history, physical exam, and lab tests. This does not guarantee enrolment.

Eligibility at a Glance

Key info

  • Age: 18 Years and over
  • Who can join: All genders

What the study is looking for

  • ✓Subject gives voluntary agreement to take part to participate in the study.
  • ✓Subject is ≥18 years of age, inclusive, at the time of signing agreement to take part.
  • ✓Subject has radiological evidence of lung fibrosis of \>10% extent on an HRCT scan in the previous 12 months...
  • ✓Clinically significant decline in % predicted FVC based on ≥10% relative decline
  • ✓Marginal decline in % predicted FVC based on ≥5% to \<10% relative decline combined with worsening of respiratory...

Who cannot take part

  • ✗Subject is pregnant or lactating.
  • ✗Subject has primary obstructive airway physiology (forced expiratory volume in 1 second/FVC \<0.70 at Screening) or...
  • ✗Subject has a diagnosis of IPF.
  • ✗Subject has shown intolerance or significant lack of efficacy to a prostacyclin or prostacyclin analogue that...
  • ✗Subject is receiving \>10 L/min of oxygen supplementation by any mode of delivery at rest at Baseline.
See the full 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), 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.

Where Is This Study? (6 UK sites)

Birmingham Heartlands Hospital

Birmingham, United Kingdom

Recruiting
Site contact (verified)
Christopher Huntley, MDPrincipal Investigator

Royal Papworth Hospital

Cambridge CB2 0AY, United Kingdom

Recruiting
Site contact (verified)
Christine Fiddley, MDPrincipal Investigator

NHS Lothian, Royal INfirmary of Edingburgh

Edinburgh EH16 4SA, United Kingdom

Recruiting
Site contact (verified)
Nikhil Hirani, MDPrincipal Investigator

Guy's Hospital

London SE1 9RT, United Kingdom

Recruiting
Site contact (verified)
Katherine Myall, MDPrincipal Investigator

Manchester University NHS Foundation Trust, North Manchester General Hospital

Manchester M8 5RB, United Kingdom

Recruiting
Site contact (verified)
Zoe Borril, MDPrincipal Investigator

NHS Tayside, Perth Royal Infirmary

Perth PH1 1NX, United Kingdom

Recruiting
Site contact (verified)
Mark Spears, MDPrincipal Investigator

How to Get in Touch

United Therapeutics Global Medical Information

Sponsor contact

CONTACT

919-485-8350 clinicaltrials@unither.com
Data sourced from ClinicalTrials.gov · Last verified: 2026-07