Tyvaso (treprostinil) / United Therapeutics Corp - LARVOL DELTA

TETON: Study of Efficacy and Safety of Inhaled Treprostinil in Subjects With Idiopathic Pulmonary Fibrosis (clinicaltrials.gov) - P3 | N=576 | Active, not recruiting | Sponsor: United Therapeutics | Trial completion date: Jun 2025 ➔ Jan 2026 | Trial primary completion date: Jun 2025 ➔ Jan 2026

Trial completion date • Trial primary completion date • Idiopathic Pulmonary Fibrosis • Immunology • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases

United Therapeutics Corporation Reports Record Second Quarter 2025 Financial Results (Businesswire) - "Tyvaso DPI record total revenue of $315 million, reflecting 22 percent growth over the second quarter of 2024....Double-digit, year-over-year revenue growth for Nebulized Tyvaso, Orenitram, and Unituxin with record quarterly revenue for Orenitram; Phase 3 TETON 2 study of Nebulized Tyvaso in idiopathic pulmonary fibrosis is complete; data expected in September 2025; phase 3 TETON 1 data expected in the first half of 2026; Enrollment for the phase 3 ADVANCE OUTCOMES study of ralinepag in pulmonary arterial hypertension is complete; data expected in the first half of 2026."

Enrollment closed • P3 data • Sales • Idiopathic Pulmonary Fibrosis • Neuroblastoma • Neuroendocrine Tumor • Pulmonary Arterial Hypertension

Practical Considerations for Managing Patients on Tyvaso DPI (Treprostinil Inhalation Powder). (PubMed, Pulm Circ) - "This review provides an overview of inhaled prostacyclin therapy with a focus on practical considerations for managing PAH and PH-ILD patients treated with Tyvaso DPI. Recommendations from a panel of pulmonary hypertension advanced practice providers include patient selection, education, communication, onboarding and monitoring, transition and titration, side effect mitigation, and the availability of clinician- and patient-facing resources."

Journal • Review • Cardiovascular • Hypertension • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

SAPPHIRE: Inhaled Treprostinil in Sarcoidosis Patients With Pulmonary Hypertension (clinicaltrials.gov) - P2 | N=10 | Recruiting | Sponsor: University of Florida | Trial completion date: Jul 2025 ➔ Jul 2026 | Trial primary completion date: Jan 2025 ➔ Dec 2025

Trial completion date • Trial primary completion date • Cardiovascular • Hypertension • Immunology • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Sarcoidosis

Inhaled Treprostinil (Tyvaso Nebulizer) For COPD Patients With Hypoxemia (clinicaltrials.gov) - P1 | N=10 | Not yet recruiting | Sponsor: Yuh Chin T Huang, MD, MHS

New P1 trial • Chronic Obstructive Pulmonary Disease • Immunology • Pulmonary Disease • Respiratory Diseases

Transitioning From Parenteral Treprostinil to LIQ861 in a Patient With PAH (ATS 2025) - "This is the first case report describing transitioning a patient with PAH from intravenous treprostinil to LIQ861.Case description:Herein, we present a case of a 42-year-old female with idiopathic PAH, oxygen dependent, initially NYHA II, who was enrolled in the INSPIRE study on August 23, 2018 and transitioned from Tyvaso 12 breaths QID to LIQ861 79.5ug QID in combination with sildenafil and macitentan. On Day 2174, NYHA II, 6MWD 320 meters in combination with LIQ861 212ug QID, Ambrisentan 10mg and Sildenafil 40mg TID. Inhaled Treprostinil therapy can offer comparable efficacy to parenteral routes in the management of PAH, while reducing systemic side effects and eliminating the need for subcutaneous or intravenous access. To our knowledge, this is the first case report of transitioning between LIQ861 and I.V. Treprostinil."

Clinical • Cardiovascular • Congestive Heart Failure • Heart Failure • Pain

Teton Phase 3 Clinical Trials of Inhaled Treprostinil in the Treatment of Idiopathic Pulmonary Fibrosis: Annual Update of Preliminary Baseline Data (ATS 2025) - P3 | "Stable background use of pirfenidone or nintedanib is allowed... The prospectively designed TETON Phase 3 clinical trials aim to definitively explore the hypothesis around inhaled treprostinil in IPF and may offer a much-needed inhaled treatment option for this vulnerable patient population."

Clinical • P3 data • Cardiovascular • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

An Ascent to Week 8: Initial Safety and Exploratory Efficacy Data on LIQ861 Dry Powder Inhaled Treprostinil in Ph-ILD Patients (ATS 2025) - P | "This abstract is funded by: Liquidia Technologies Inc. Inhaled LIQ861 was well tolerated for up to 8 weeks in the first 13 patients. With a single exception, all treatment related TEAEs were mild, and none were dose dependent. The median dose was 132.5 mcg QID and 33.3% of patients achieved a dose of ≥159 mcg QID (equivalent to ≥18 breaths of TYVASO)."

Clinical • Cough • Immunology • Infectious Disease • Interstitial Lung Disease • Pain • Pneumonia

The Minimal Important Difference in NT-proBNP in Various Forms of Pulmonary Hypertension (ATS 2025) - " Data from two registration trials were used to determine the MID for PAH (FREEDOM-EV: oral treprostinil versus placebo) and PH-ILD (INCREASE: inhaled treprostinil versus placebo)... The estimated MID for NT-proBNP change in PAH and PH-ILD is remarkably similar -36% and -38%, respectively. Failure to achieve the MID for NT-proBNP change is strongly associated with clinically important outcomes in both PAH and PH-ILD, demonstrating that measuring NT-proBNP change over 12-16 weeks can be informative for prognosis and may be useful for decisions regarding treatment escalation in both PAH and PH-ILD."

Cardiovascular • Idiopathic Pulmonary Fibrosis • Immunology • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Rheumatology • NPPB

TETON-PPF Clinical Trial of Inhaled Treprostinil for the Treatment of Progressive Pulmonary Fibrosis: Preliminary Baseline Demographics (ATS 2025) - P3 | "Background use of pirfenidone or nintedanib is allowed... The prospectively designed TETON-PPF Phase 3 clinical trial is being performed in parallel with the TETON studies for IPF to avoid any delay in this potential treatment option becoming available for PPF patients, who currently have limited treatment options but a similar prognosis as IPF patients."

Clinical • Cardiovascular • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Impact of Pharmacist Managed Pulmonary Arterial Hypertension Program on TYVASO Adherence (ISPOR 2025) - "OBJECTIVES: Pulmonary Arterial Hypertension (PAH) is a chronic condition requiring consistent medication adherence to optimize outcomes. Enrollment in the PAH program was associated with improved treprostinil adherence. These preliminary results highlight the potential of targeted pharmacy programs to enhance medication adherence and patient outcomes in PAH."

Adherence • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

United Therapeutics Corporation Reports First Quarter 2025 Financial Results (Businesswire) - "Total Tyvaso revenues grew by 25 percent to $466.3 million in the first quarter of 2025, compared to $372.5 million in the first quarter of 2024....The growth in Tyvaso DPI revenues resulted primarily from an increase in quantities sold of $97.4 million and, to a lesser extent, a price increase, partially offset by higher gross-to-net deductions."

Sales • Interstitial Lung Disease • Pulmonary Arterial Hypertension

Safety of Low-Dose Oral Treprostinil in PH-HFpEF Supports Use in Patients with PAH with Cardiovascular Comorbidities (ISHLT 2025) - P3 | "Recent analyses of the FREEDOM-EV and TRIUMPH trials demonstrated no difference in treatment response or tolerability of oral and inhaled treprostinil (TRE) with increasing CV comorbidity burden...No oral TRE patients died during the study; one (2%) PBO patient died during the study.Conclusion In SOUTHPAW, AEs were consistent with prior TRE studies, and there were no concerning safety signals with low doses of oral TRE (study maximum dose 4 mg TID). This suggests that oral TRE may be safely used in PAH patients with CV comorbidities and risk factors for HFpEF."

Clinical • Cardiovascular • Congestive Heart Failure • Fatigue • Heart Failure • Pain • Pulmonary Disease

Heart Lung Transplantation for Fibrosing Mediastinitis (ISHLT 2025) - "While awaiting transplant, he was on dobutamine, tyvaso, diuretics & HFNC...Rituximab, Vascular & bronchoscopic stents and surgical corrections are options. Glucocorticoids & antifungals are not beneficial. Heart lung transplantation was successful in our case and may be considered."

Cardiovascular • Cough • Fibrosis • Pulmonary Arterial Hypertension • Pulmonary Disease • Rare Diseases • Renal Disease • Respiratory Diseases • Transplantation

Derivation of a simple risk calculator for predicting clinical worsening in patients with pulmonary hypertension due to interstitial lung disease. (PubMed, JHLT Open) - "The INCREASE study was a 16-week study that evaluated inhaled treprostinil in patients with ILD-PH...The risk calculators may also find utility as part of the composite allocation score of ILD-PH patients listed for lung transplant. Future research in this area could include incorporating longer-term outcomes as well as validating the risk models in a separate patient population."

Journal • Cardiovascular • Hypertension • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Transplantation

Tyvaso, a treatment for pulmonary hypertension, re-challenges for listing as an 'essential treatment drug' [Google translation] (HIT News) - "'Tyvaso Inhalation Solution (ingredient: treprostinil),' a treatment for pulmonary hypertension associated with interstitial lung disease (PH-ILD), has reapplied for reimbursement as an essential drug for medical treatment. As the scope of use has been adjusted for reapplication, attention is being paid to whether it will be listed on the essential drug track, which has effectively become a dead letter....In order to be included in the essential medicine track, all of the following conditions must be met: there must be no alternative treatment, life-threatening serious disease, rare disease, etc., targeting a small number of patients, and demonstrating clinically meaningful improvement. Therefore, the essential medicine status has become meaningless since 2015."

Reimbursement • Interstitial Lung Disease • Pulmonary Arterial Hypertension

TETON: Study of Efficacy and Safety of Inhaled Treprostinil in Subjects With Idiopathic Pulmonary Fibrosis (clinicaltrials.gov) - P3 | N=576 | Active, not recruiting | Sponsor: United Therapeutics | Recruiting ➔ Active, not recruiting

Enrollment closed • Idiopathic Pulmonary Fibrosis • Immunology • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases

United Therapeutics Corporation Reports Fourth Quarter and Full Year 2024 Financial Results (Businesswire) - "'both TETON studies in idiopathic pulmonary fibrosis are enrolled, facilitating data starting the second half of this year; ralinepag, our potentially best-in-class once-daily oral prostacyclin agonist, will generate data next year'...Total Tyvaso revenues grew by 19 percent to $415.9 million in the fourth quarter of 2024, compared to $350.6 million in the fourth quarter of 2023...Total Tyvaso revenues grew by 31 percent to $1,620.4 million in 2024, compared to $1,233.7 million in 2023...The growth in Remodulin revenues resulted from an increase in U.S. Remodulin revenues, driven by an increase in quantities sold. The growth in Orenitram revenues resulted from an increase in quantities sold and, to a lesser extent, a price increase."

Clinical data • Commercial • P3 data • Idiopathic Pulmonary Fibrosis • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Respiratory Diseases

A Study of Treprostinil to Treat Interstitial Lung Disease Pulmonary Hypertension (clinicaltrials.gov) - P2 | N=29 | Completed | Sponsor: Mayo Clinic | Enrolling by invitation ➔ Completed

Trial completion • Cardiovascular • Hypertension • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

United Therapeutics Corporation Announces Full Enrollment of the TETON 1 Study of Inhaled Treprostinil for the Treatment of Idiopathic Pulmonary Fibrosis (Businesswire) - "United Therapeutics Corporation...announced full enrollment of the TETON 1 study evaluating the use of Tyvaso (treprostinil) inhalation solution (nebulized Tyvaso) for the treatment of idiopathic pulmonary fibrosis (IPF). The TETON 1 study enrolled 598 patients and is part of the three-study global TETON clinical trial program evaluating the use of inhaled treprostinil in IPF and a similar condition, progressive pulmonary fibrosis (PPF)....Enrollment in TETON 2 completed in July 2024 and top-line data for that study is expected in the second half of 2025....The TETON 1 study is a 598-patient, multicenter, randomized, double-blind, placebo-controlled phase 3 registration study to evaluate the safety and efficacy of nebulized Tyvaso in subjects with IPF over a 52-week period at sites in the United States and Canada. The study reached full enrollment in January 2025 and top-line data is expected in the first half of 2026."

Enrollment closed • P3 data: top line • Idiopathic Pulmonary Fibrosis

United Therapeutics Corporation to Feature Clinical Data Across its Commercial and Development Portfolio at the Pulmonary Vascular Research Institute 2025 Annual Congress (Businesswire) - "United Therapeutics Corporation...announced that five posters across its commercial and development portfolio in pulmonary hypertension (PH) will be presented at the Pulmonary Vascular Research Institute (PVRI) 2025 Annual Congress taking place January 29 through February 1, 2025 in Rio de Janeiro. In addition, professionals from United Therapeutics will participate in two speaking events associated with the congress."

Clinical data • Pulmonary Arterial Hypertension

A Study of Treprostinil to Treat Interstitial Lung Disease Pulmonary Hypertension (clinicaltrials.gov) - P2 | N=24 | Enrolling by invitation | Sponsor: Mayo Clinic | Trial completion date: Oct 2024 ➔ Jan 2025 | Trial primary completion date: Oct 2024 ➔ Jan 2025

Trial completion date • Trial primary completion date • Cardiovascular • Hypertension • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Treatment with Oral or Inhaled Treprostinil in Patients with Pulmonary Arterial Hypertension and Cardiovascular Comorbidities. (PubMed, Chest) - "This post hoc analysis suggests that patients with PAH and cardiovascular comorbidities can benefit from combination therapy with inhaled or oral treprostinil."

Journal • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • NPPB

Patents delisted for Tyvaso from Orange Book (Orange Book) - Patent no.s 10716793 and 9604901

Patent • Idiopathic Pulmonary Fibrosis

United Therapeutics Corporation Reports Third Quarter 2024 Financial Results (Businesswire) - "Total Tyvaso revenues grew by 33 percent to $433.8 million in the third quarter of 2024, compared to $325.8 million in the third quarter of 2023. This growth was primarily due to an increase in quantities sold, driven by the commercial launch of Tyvaso DPI in June 2022 and continued growth in commercial utilization by patients with pulmonary hypertension associated with interstitial lung disease and, to a lesser extent, price increases."

Commercial • Idiopathic Pulmonary Fibrosis