Yutrepia (treprostinil inhaled dry powder) / Liquidia Technologies - LARVOL DELTA

Efficacy of inhaled treprostinil versus PDE5 inhibitors and no treatment in ILD-PH (ERS 2025) - No abstract available

Clinical • Interstitial Lung Disease

Real-life use of inhaled treprostinil in pulmonary hypertension associated with interstitial lung disease (ERS 2025) - No abstract available

Clinical • Cardiovascular • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Inhaled Treprostinil treatment in pulmonary hypertension associated with interstitial lung disease in an outermost region of Europe. (ERS 2025) - No abstract available

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

TETON-PPF clinical trial of inhaled treprostinil for the treatment of progressive pulmonary fibrosis (ERS 2025) - No abstract available

Clinical • Immunology • Pulmonary Disease • Respiratory Diseases

TETON phase 3 clinical trials of inhaled treprostinil in the treatment of idiopathic pulmonary fibrosis (ERS 2025) - No abstract available

Clinical • P3 data • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases

Liquidia gets $50M more after first sale of Yutrepia to treat PH (Pulmonary Hypertension News) - "Following the first commercial sale of Yutrepia (treprostinil) inhalation powder to treat certain types of pulmonary hypertension, the therapy’s maker, Liquidia, has received an additional $50 million under its financing agreement with Healthcare Royalty (HCRx)...Per that HCR Agreement, Liquidia has been raising funds by selling HCRx a percentage of future revenue generated from drug sales...To date, Liquidia has secured $175 million of the $200 million in total potential funding, per the release. An additional $25 million remains available if Liquidia achieves net sales of Yutrepia over $100 million at any time on or before June 30, 2026. The extra $50 million paid by HCRx is subject to a fixed payment schedule through 2033."

Financing • Sales • Pulmonary Arterial Hypertension

EFFICACY AND SAFETY OF INHALED TREPROSTINIL IN GROUP 3 PULMONARY HYPERTENSION: A SYSTEMATIC REVIEW (CHEST 2025) - No abstract available

Clinical • Review • Cardiovascular • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

LEVEL THREE CARDIOPULMONARY EXERCISE TESTING AS A DECISION-MAKING TOOL FOR INHALED TREPROSTINIL THERAPY IN NONSEVERE PULMONARY HYPERTENSION-INTERSTITIAL LUNG DISEASE (CHEST 2025) - No abstract available

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

DEVELOPMENT AND VALIDATION OF A SCORING SYSTEM FOR PREDICTION OF TOLERANCE TO INHALED TREPROSTINIL IN PATIENTS WITH PAH OR PH-ILD (CHEST 2025) - No abstract available

Clinical • Interstitial Lung Disease

IMPACT OF INHALED TREPROSTINIL INITIATION TIMING ON HOSPITALIZATIONS IN PATIENTS WITH PULMONARY HYPERTENSION DUE TO INTERSTITIAL LUNG DISEASE (CHEST 2025) - No abstract available

Clinical • Cardiovascular • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

1032: PH-ILD Controversies: A Pro-Con Debate (CHEST 2025) - "2. All patients with PH-ILD should be treated with inhaled treprostinil"

Interstitial Lung Disease

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

U.S. FDA Approves Liquidia’s YUTREPIA (treprostinil) Inhalation Powder for Patients with Pulmonary Arterial Hypertension (PAH) and Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) (GlobeNewswire) - "Liquidia Corporation...announced today that the U.S. Food and Drug Administration (FDA) has approved YUTREPIA (treprostinil) inhalation powder, a prostacyclin analog for adults with pulmonary arterial hypertension (PAH) and pulmonary hypertension associated with interstitial lung disease (PH-ILD) to improve exercise ability....The approval of YUTREPIA is based on findings from the Phase 3 INSPIRE trial which evaluated patients who were naïve to treprostinil, as well as those transitioning to YUTREPIA from nebulized treprostinil."

FDA approval • Hypertension • Interstitial Lung Disease • Pulmonary Arterial Hypertension

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

Enhancing Diagnostic Precision: Characterizing Subgroup Variability Within WHO Group 3 Pulmonary Hypertension Using ICD-10 Code I27.23 and Manual EHR Review (ATS 2025) - "Additionally, data on inhaled treprostinil, PDE5 inhibitors, and antifibrotic treatments were extracted. The diversity of underlying PH disorders observed in patients the I27.23 code highlights the need for more specific ICD-10 coding to facilitate accurate identification and research of Group 3 PH subgroups. Enhanced classification may improve patient care and support targeted research initiatives."

Review • Cardiovascular • Chronic Obstructive Pulmonary Disease • Immunology • Interstitial Lung Disease • Obstructive Sleep Apnea • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Rheumatology • Sleep Disorder

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

Shifting Paradigms: Exploring Super-responders to Prostacyclin Therapy in Systemic Sclerosis-associated Pulmonary Arterial Hypertension - A Case Series and Review of Literature (ATS 2025) - "All received prostacyclin therapy: two subcutaneous Treprostinil, one inhaled Treprostinil, and one started subcutaneously before transitioning to oral medication... Although 'super-responders to prostacyclin' is not a recognized clinical entity yet, variability in response to this drug class has been observed in clinical trials, with a subset of patients achieving long-term survival and even near normalization of hemodynamics, as observed in our patient cohort. Current studies speculate that prostacyclin therapy not only causes pulmonary artery vasodilation, but also influences vascular remodeling process by potentially recruiting endothelial progenitor cells (EPCs) into the pulmonary vasculature, as well as reducing the number and procoagulant activity of platelet micro-vesicles (PMVs). It will be worthwhile to investigate EPC count and PMVs as surrogate markers for prostacyclin response to address treatment response heterogeneity."

Clinical • Review • Cardiovascular • Immunology • Pulmonary Arterial Hypertension • Respiratory Diseases • Scleroderma • Systemic Sclerosis

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

Maternal Pulmonary Arterial Hypertension Treated With IV Epoprostenol - A Case Report (ATS 2025) - "Her pre-gestation medications included Ambrisentan, Selexipag, and Sildenafil...The patient was then transitioned from IV Epoprostenol to inhaled Treprostinil via dry powder inhaler...IV Epoprostenol may be used adjunctively in the management of pregnant PAH patients and could improve peri and postpartum outcomes. This case adds to a growing literature base on this subject; the difficulties of performing a blinded, randomized clinical trial in this cohort are acknowledged."

Case report • Clinical • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

Comparing Pulmonary Arterial Hypertension Care in Rural and Urban Settings: Similarities, Surprises, and Treatment Trends (ATS 2025) - "This study suggests that urban-rural disparities in the clinical course of PAH may not be as pronounced as previously thought. Both groups demonstrated similar baseline characteristics, diagnostic testing, and treatment outcomes. Contrary to expectations, virtual visit usage was comparable between populations, potentially reflecting the improvements in virtual care management due to the COVID-19 pandemic."

Cardiovascular • Infectious Disease • Novel Coronavirus Disease • Pulmonary Arterial Hypertension • Respiratory Diseases

Efficacy and Safety of Selexipag in Connective Tissue Disease-Associated Pulmonary Arterial Hypertension With Concomitant Interstitial Lung Disease (ATS 2025) - "Pulmonary vasodilators have long been unsuccessfully studied in PH-ILD patients until the INCREASE trial demonstrated the effectiveness of inhaled Treprostinil in improving exercise capacity in this subgroup of Group 3 PH, highlighting the potential of the prostacyclin pathway to benefit these patients without worsening oxygenation. While other systemic agents used in treating PAH have not demonstrated a similar benefit as inhaled Treprostinil in treatment of PH-ILD, our study highlights the potential role of Selexipag in patients with concomitant CTD-PAH and parenchymal lung disease. Selexipag demonstrated statistically significant improvements in exercise capacity, as well as reductions in eRVSP; importantly, there was no evidence of worsening ventilation-perfusion mismatch in this cohort."

Clinical • Cardiovascular • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Rheumatology

Evaluation of Screening for Pulmonary Hypertension Associated With Chronic Respiratory Disease (ATS 2025) - "With the advent of inhaled treprostinil, early diagnosis of CLD-PH has become even more crucial, necessitating simple and accurate screening tests. However, cutoffs and diagnostic accuracy varied significantly by disease, showing distinct trends in COPD vs. ILD/CPFE. A PA/Ao ratio >1 demonstrated high specificity (83.3-91.7%) across CLD types, further improving when combined with other diagnostic tests.[Conclusions]The accuracy of screening for CLD-PH depends on the underlying CLD, but a combination of several widely available tests can potentially increase diagnostic effectiveness at any hospital."

Cardiovascular • Chronic Obstructive Pulmonary Disease • Immunology • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Changes in Cardiac Effort in Pulmonary Hypertension-Interstitial Lung Disease: Insights From the Ascent Trial (ATS 2025) - P | "This abstract is funded by: Liquidia Technologies RATIONALE: The ASCENT study [NCT06129240], Cohort A, is a prospective, multicenter, open-label trial evaluating the safety, tolerability, and efficacy of LIQ861, an inhaled dry powder formulation of treprostinil, in patients with pulmonary hypertension-interstitial lung disease (PH-ILD), including combined pulmonary fibrosis and emphysema (CPFE). The ASCENT trial demonstrates that Cardiac Effort shows early insight into physiologic improvements in PH-ILD after treatment with LIQ861. Incorporating continuous electrocardiogram heart rate monitoring may provide additive and complementary insight into the physiology of the patient's response to treatment relative to the 6MWD in isolation.Figure 1a) Change in Cardiac Effort from Baseline to Week 8. 1b) Cardiac Effort Week 8 versus Baseline."

Cardiovascular • Immunology • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Liquidia Corporation Provides Update on Litigation Filed by United Therapeutics (GlobeNewswire) - "Liquidia Corporation...announced that United Therapeutics Corporation (UTHR) filed a complaint on May 9, 2025, in the U.S. District Court for the Middle District of North Carolina (Case No. 1:25-cv-00368) against Liquidia alleging infringement of U.S. Patent No. 11,357,782 (the ‘782 patent). Additionally, the complaint seeks to enjoin Liquidia from commercializing YUTREPIA (treprostinil) inhalation powder if approved by the U.S. Food and Drug Administration (FDA) to treat pulmonary arterial hypertension (PAH) and pulmonary hypertension associated with interstitial lung disease (PH-ILD)....The ‘782 patent, which issued on June 14, 2022, arises out of the same patent family as U.S. Patent No. 10,716,793 (the ‘793 patent) and claims the same general method of administering inhaled treprostinil to pulmonary hypertension patients. As disclosed in July 2022, the ‘793 patent was held to be invalid in a proceeding before the Patent Trial and Appeal Board (PTAB)."

Patent • Interstitial Lung Disease • Pulmonary Arterial Hypertension