Tyvaso (treprostinil) / United Therapeutics Corp - LARVOL DELTA

Real-World Comparison of Patients With PH-ILD Initiating Inhaled Treprostinil Versus Patients Who Remain Untreated. (PubMed, Pulm Circ) - "Patients who initiated inhaled treprostinil had a 30% decreased risk of hospitalization compared to untreated patients (relative risk: 0.70; 95% CI: 0.59-0.83; p < 0.01). Among real-world patients with PH-ILD, treatment with inhaled treprostinil is associated with fewer all-cause hospitalizations and ICU-related hospitalizations compared to untreated patients with PH-ILD."

Journal • Real-world evidence • Cardiovascular • Hypertension • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

United Therapeutics Corporation Reports Record Third Quarter 2025 Financial Results (United Therapeutics Press Release) - "Total Tyvaso revenues grew by 10 percent to $478.0 million in the third quarter of 2025, compared to $433.8 million in the third quarter of 2024...Total Tyvaso revenues grew by 10 percent to $478.0 million in the third quarter of 2025, compared to $433.8 million in the third quarter of 2024...The growth in Orenitram revenues resulted primarily from an increase in quantities sold of $11.7 million and, to a lesser extent, a price increase."

Commercial • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Arterial Hypertension

Inhaled Treprostinil (Tyvaso Nebulizer) For COPD Patients With Hypoxemia (clinicaltrials.gov) - P2 | N=10 | Recruiting | Sponsor: Bastiaan Driehuys | Phase classification: P1 ➔ P2

Phase classification • Chronic Obstructive Pulmonary Disease • Immunology • Pulmonary Disease • Respiratory Diseases

Total Tyvaso product revenues grew 10% to $478 million, with Tyvaso DPI sales surging 22% to $336.2 million, partially offset by an 11% decline in nebulized Tyvaso (Investing.com)

Sales • Interstitial Lung Disease • Pulmonary Arterial Hypertension

Inhaled Treprostinil (Tyvaso Nebulizer) For COPD Patients With Hypoxemia (clinicaltrials.gov) - P1 | N=10 | Recruiting | Sponsor: Bastiaan Driehuys | Not yet recruiting ➔ Recruiting | Initiation date: Jul 2025 ➔ Sep 2025

Enrollment open • Trial initiation date • Chronic Obstructive Pulmonary Disease • Immunology • Pulmonary Disease • Respiratory Diseases

MannKind Completes Acquisition of scPharmaceuticals, Accelerating Revenue Growth in Cardiometabolic Care (Mannkind Corp Press Release) - "The transaction will strengthen MannKind’s commercial and medical capabilities by integrating scPharmaceuticals’ experienced team into its existing infrastructure. MannKind is positioned as a diversified, growth-focused biopharmaceutical company with its commercial assets—Afrezza, FUROSCIX and V-Go -- along with Tyvaso DPI-related revenues, contributing to an annualized run rate of over $370 million based on Q2 2025 results. Additionally, the FUROSCIX ReadyFlow Autoinjector supplemental New Drug Application (sNDA) filing was submitted as planned in Q3 2025."

M&A • Pulmonary Arterial Hypertension • Type 2 Diabetes Mellitus

United Therapeutics Corporation Announces TETON-2 Pivotal Study of Tyvaso Meets Primary Endpoint for the Treatment of Idiopathic Pulmonary Fibrosis (Businesswire) - "Tyvaso demonstrated superiority over placebo for the change in absolute FVC by 95.6 mL (Hodges-Lehmann estimate, p <0.0001) from baseline to week 52 in patients with IPF. Benefits of Tyvaso were observed across all subgroups, such as use of background therapy (nintedanib, pirfenidone, or no background therapy), smoking status, and supplemental oxygen use."

P3 data • Idiopathic Pulmonary Fibrosis

United Therapeutics Corporation to Review Data from the Successful TETON-2 Pivotal Study of Nebulized Tyvaso in Idiopathic Pulmonary Fibrosis - "During the webcast, Steven D. Nathan, M.D., Schar Chair, Advanced Lung Disease and Lung Transplant Program at Inova Fairfax Hospital and Chair of the TETON Steering Committee, will review data that were presented earlier in the day at the European Respiratory Society Congress."

Trial status • Idiopathic Pulmonary Fibrosis

TETON-OLE: Extension Study of Inhaled Treprostinil in Subjects With Fibrotic Lung Disease (clinicaltrials.gov) - P3 | N=1850 | Enrolling by invitation | Sponsor: United Therapeutics | N=792 ➔ 1850

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

BREEZE Optional Extension Phase: Long-Term Safety and Efficacy of Treprostinil Dry Powder Inhaler (Tyvaso DPI) in Pulmonary Arterial Hypertension. (PubMed, Respir Med) - P1 | "The BREEZE OEP successfully demonstrated the safety of long-term treatment with treprostinil DPI in patients with PAH."

Journal • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • 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

Effects of Inhaled Treprostinil on Exercise Performance in Exercise Induced Pulmonary Hypertension (clinicaltrials.gov) - P1 | N=8 | Not yet recruiting | Sponsor: University of California, San Diego

New P1 trial • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

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

TETON-2: Multinational Study of Efficacy and Safety of Inhaled Treprostinil in Subjects With Idiopathic Pulmonary Fibrosis (clinicaltrials.gov) - P3 | N=597 | Completed | Sponsor: United Therapeutics | Active, not recruiting ➔ Completed | Trial completion date: Jan 2026 ➔ Jun 2025 | Trial primary completion date: Jan 2026 ➔ Jun 2025

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

TETON-2: Multinational Study of Efficacy and Safety of Inhaled Treprostinil in Subjects With Idiopathic Pulmonary Fibrosis (clinicaltrials.gov) - P3 | N=597 | Active, not recruiting | Sponsor: United Therapeutics | Trial completion date: Jul 2025 ➔ Jan 2026 | Trial primary completion date: Jul 2025 ➔ Jan 2026

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

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