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

Decluttering Complexity: A Pragmatic Framework for Simplifying Global Health Economic Models for HTA (ISPOR-EU 2025) - "The methodology is illustrated using Ferrer's inhaled treprostinil model for pulmonary hypertension associated with interstitial lung disease. We analyzed the existing combined CE and BI model to identify sources of complexity, including redundant inputs, dispersed settings across multiple sheets, and intricate data flows. This framework enables structured simplification of global health economic models without compromising robustness. It enhances usability and adaptability for global HTA needs. Further research could refine the methodology through broader use cases, and stakeholder-informed criteria."

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

Pulmonary arterial hypertension and the limits of convenience: a critical look at yutrepia. (PubMed, Ann Med Surg (Lond)) - No abstract available

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

SAFETY AND EXPLORATORY EFFICACY DATA OF LIQ861 DRY POWDER INHALED TREPROSTINIL IN PH-ILD PATIENTS: ASCENT TO WEEK 16 (CHEST 2025) - P | "In PH-ILD patients, inhaled LIQ861 was well tolerated over 16 weeks, with the majority titrating to doses $159 mcg, which is equivalent to $18 breaths of nebulized treprostinil, QID by Week 16. This represents a higher dose equivalent than was seen in clinical trials of nebulized treprostinil in PAH and PH-ILD. A notable improvement of +31.5 meters was observed in this 'real-world population' of PH-ILD patients."

Clinical • Late-breaking abstract • Cardiovascular • Cough • Immunology • Infectious Disease • Inflammation • Interstitial Lung Disease • Pneumonia • Pulmonary Arterial Hypertension

7100: NOW APPROVED YUTREPIA™(treprostinil) Inhalation Powder : Advancing the Power of Prostacyclins in PAH and PH-ILD (CHEST 2025) - "Description This presentation will explore YUTREPIA: the first and only dry powder formulation of treprostinil enabled by PRINT technology, delivered through a low-effort device, for the treatment of patients with PAH and PH-ILD. See full prescribing information at YUTREPIA (treprostinil) inhalation powder; HCP Website - www.yutrepiahcp.com."

Interstitial Lung Disease

PRECISION OVER PROCEDURE: TAILORING PULMONARY HYPERTENSION TREATMENT IN SCIMITAR SYNDROME WITH LUNG HYPOPLASIA (CHEST 2025) - "The patient was started on inhaled Treprostinil dry powder inhaler and was titrated to 48 mcg QID. This case demonstrates need for nuanced approach to multifactorial mixed PH with complex anatomy and physiology. Scimitar syndrome, associated with other congenital anomalies including pulmonary hypoplasia, necessitates an individualized management approach."

Cardiovascular • Gastroenterology • Genetic Disorders • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

EFFICACY AND SAFETY OF INHALED TREPROSTINIL IN GROUP 3 PULMONARY HYPERTENSION: A SYSTEMATIC REVIEW (CHEST 2025) - "Inhaled treprostinil showed positive results in improving functional outcomes for patients with PH related to ILD, but its role in PH-COPD remains uncertain due to variable results. More studies are needed to assess the long-term safety and tolerability of inhaled treprostinil, particularly in patients with pulmonary hypertension due to COPD. CLINICAL IMPLICATIONS: Our systematic review findings contribute to the growing body of evidence on inhaled treprostinil for Group 3 pulmonary hypertension."

Clinical • Review • Cardiovascular • Chronic Obstructive Pulmonary Disease • Cough • Immunology • Interstitial Lung Disease • 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) - "Both cohorts consisted of non-severe PH-ILD patients with no significant differences in baseline characteristics (all p>0.05), yet their exercise responses diverged. Although non-severe PH-ILD is known to eventually progress, the static PVR group that did not receive treatment showed no significant progression over the 16-week period, maintaining stable parameters at follow-up. Moreover, by utilizing Level 3 CPET, we were able to identify patients likely to respond to therapy, and targeted intervention in these individuals with inhaled treprostinil led to significant improvements."

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

IMPACT OF INHALED TREPROSTINIL INITIATION TIMING ON HOSPITALIZATIONS IN PATIENTS WITH PULMONARY HYPERTENSION DUE TO INTERSTITIAL LUNG DISEASE (CHEST 2025) - "In real world claims data, timely initiation of inhaled treprostinil is associated with a reduction in all-cause hospitalizations in patients with pulmonary hypertension due to interstitial lung disease. CLINICAL IMPLICATIONS: Earlier initiation of inhaled treprostinil could potentially reduce hospitalizations in patients with pulmonary hypertension due to interstitial lung disease."

Clinical • 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) - "The ITIS provides a simple, non-invasive tool for predicting intolerance to inhaled treprostinil based on routine clinical and hemodynamic assessments. Patients with right ventricular dysfunction, concomitant obstructive lung physiology, and advanced functional class were at higher risk of treatment intolerance. The scoring system demonstrated high predictive accuracy, particularly in PAH patients, reinforcing the importance of these parameters in guiding treatment decision- making."

Clinical • Cardiovascular • Heart Failure • Interstitial Lung Disease • Pulmonary Arterial Hypertension

CHANGES IN CARDIAC EFFORT IN PULMONARY HYPERTENSION-INTERSTITIAL LUNG DISEASE: INSIGHTS FROM THE ASCENT TRIAL (CHEST 2025) - P | "PURPOSE: 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 highlights that Cardiac Effort changes and provides early insights into physiological improvements in patients with PH-ILD following treatment with LIQ861. CLINICAL IMPLICATIONS: The integration of continuous electrocardiogram heart rate monitoring offers additional and complementary insights into the patient's physiological response to treatment beyond what the 6MWD alone can reveal."

Cardiovascular • Immunology • 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

Volatile Organic Compound Exposure, Scleroderma-Spectrum Autoimmunity, and Pulmonary Arterial Hypertension: A Case Report (HFSA 2025) - "He was initiated on sildenafil and inhaled treprostinil, with diuretics and rate control for AF. This case supports a link between chronic VOC exposure, immune dysregulation, and autoimmune-mediated PAH. In cases of unexplained PH with autoimmune features, environmental exposure history is crucial. Early identification of SSc-spectrum PAH and timely initiation of targeted therapy may alter the trajectory of this progressive condition."

Case report • Clinical • Atrial Fibrillation • Bladder Cancer • Cardiovascular • Chronic Kidney Disease • Fibrosis • Genito-urinary Cancer • Immunology • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Rheumatology • Scleroderma • Solid Tumor • Systemic Sclerosis

Efficacy of inhaled treprostinil versus PDE5 inhibitors and no treatment in ILD-PH (ERS 2025) - "Inhaled treprostinil showed greater OS versus PDE5i and no treatment, though not adjusting for all possible TEMs is a limitation of the study."

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

Pulmonary hypertension associated with chronic respiratory disease: the experience of a referral center. (ERS 2025) - "Patients with pulmonary vascular resistance (PVR) >5 WU received targeted therapy, mainly PDE-5 inhibitors (63.4%), followed by sGC (7.3%), ERA (7.3%), inhaled treprostinil (2.4%), dual therapy with ERA + PDE-5i/sGC (7.3%). One-year mortality was higher in ILD patients (21.7% vs. 5.5%, p = 0.05); 21.7% of them were untreated, accounting for 60% of deaths. Conclusions PH-CLD has a poor prognosis, but targeted therapy may benefit those with high PVR.Long-term effects remain uncertain, necessitating continued multi-disciplinary monitoring."

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

Real-life use of inhaled treprostinil in pulmonary hypertension associated with interstitial lung disease (ERS 2025) - "This study found no significant hemodynamic difference in the overall cohort. mPAP (mmHg) CI (l/min/m 2 ) PVR (WU) FVC (%) DLCO (%) 6MWD (m) NT proBNP (ng/l) V0 38.5 [33-45] 2.20 [1.9-3.0] 6.80 [5.88- 9.65] 77 [59.5- 90] 23 [19.5- 28.5] 246 [180- 332] 460 [148- 1614] V1 37.5 [30.5- 43] 2.35 [2.00- 2.63] 6.70 [5.04- 8.05] 71 [57-87] 23 [18-27] 278 [178- 345] 305.5 [111- 1190] p 0.30 0.35 0.09 0.44 0.41 0.95 0.32"

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) - "Inhaled treprostinil in PH associated with ILD was well tolerated. In our series of patients, inhaled treprostinil improved functional class and NT-proBNP levels."

Cardiovascular • Cough • Fibrosis • Immunology • 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) - "Background use of pirfenidone or nintedanib is allowed. Preliminary Baseline Characteristics of Subjects Enrolled in TETON-PPF CONCLUSIONS The TETON-PPF Phase 3 clinical trial aims to investigate inhaled treprostinil as a potential treatment option for patients with PPF. The patients recruited thus far have a slight female predominance, moderate restrictive disease, and about half are on antifibrotic therapy."

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

TETON phase 3 clinical trials of inhaled treprostinil in the treatment of idiopathic pulmonary fibrosis (ERS 2025) - "Preliminary Baseline Characteristics of Subjects Enrolled in TETON-1 and TETON-2 CONCLUSIONS The baseline study populations of the placebo-controlled, Phase 3 TETON IPF clinical trials are generally comparable to other recent trials. Headline results are expected in the second half of 2025 for the TETON-2 trial and in the first half of 2026 for the TETON-1 trial."

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

Liquidia NOW APPROVED YUTREPIA™(treprostinil) inhalation powder : Advancing the power of prostacyclins in PAH & PH-ILD (PHA-PHPN 2025) - "Sponsored by Liquidia This presentation will explore YUTREPIA™: the first and only dry powder formulation of treprostinil enabled by PRINT technology, delivered through a low-effort device, for the treatment of patients with pulmonary arterial hypertension (PAH) and pulmonary hypertension associated with interstitial lung disease (PH-ILD)."

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

Second Quarter and Recent Corporate Highlights (GlobeNewswire) - "As of August 8, specialty pharmacies reported more than 900 unique patient prescriptions leading to more than 550 patient starts. During the first six weeks post-launch, 75% of prescriptions converted to treatment starts."

Commercial • Interstitial Lung Disease • Pulmonary Arterial Hypertension

Liquidia Corporation Reports Second Quarter 2025 Financial Results and Provides Corporate Update (GlobeNewswire) - "In July and August of 2025, the company analyzed interim data from the 52-week, prospective, open-label ASCENT study which fully enrolled 54 patients with PHILD...Most patients continued on treatment to Week 16 with 10 of 54 (18.5%) discontinuing the study. There were no discontinuations stemming from drug-related adverse events, such as cough or throat irritation. Of those patients who reported a treatment related cough, 24 of 26 patients reported a mild cough and 2 patients reported a moderate cough...Dose titration remains steady, with a median dose of 132.5 mcg QID at Week 8, and 159 mcg QID at Week 16....Release of detailed clinical data is targeted for medical conferences in September and October of 2025."

Observational data • Interstitial Lung Disease

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

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