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treprostinil

✓ Approved

United Therapeutics · PTGIR · 小分子

什么是 treprostinil？

treprostinil 是一种小分子，由United Therapeutics研发。该药已获批，用于治疗相关适应症，给药途径：Injectable (Others)、Subcutaneous Injection。

药物档案

公司United Therapeutics
药物类别小分子
分子靶点PTGIR
给药途径Injectable (Others), Subcutaneous Injection
状态Approved
来源： ClinicalTrials.gov, United Therapeutics

作用机制

分子靶点

treprostinil 作用于 1 个分子靶点：

PTGIRprostaglandin I2 receptor (IP, PRIPR)
需要更深入的分析？Noah AI 可解释复杂机制并与同类药物比较。

治疗适应症

treprostinil 针对 1 个适应症，涉及 1 个治疗领域。

治疗领域疾病/病症分期
Respiratory, thoracic and mediastinal disordersPulmonary hypertension✓ Approved

相关研究文献

PubMedAdvances in respiratory medicine2026-10-26

Efficacy and Safety of Inhaled Treprostinil in Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis.

Bajpai Jyoti J, Pradhan Akshyaya A, Manek Gaurav G, Maurya Shivani S et al.

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic interstitial lung disease with high morbidity and mortality. Inhaled treprostinil showed pulmonary vascular and lung-function signals in pulmonary hypertension associated with Interstitial lung disease (PH-ILD) and was subsequently tested in IPF trials. This systematic review and meta-analysis assessed the efficacy and safety of inhaled treprostinil in adults with IPF. PubMed, Google Scholar and the Cochrane Library/CENTRAL were searched for records published from January 2000 to 2026. A random-effects meta-analysis used the DerSimonian-Laird tau squared and inverse-variance weighting methods. Continuous outcomes were pooled as generic inverse-variance effects from reported estimates and 95% confidence intervals. Hazard ratios were pooled on the log scale. Safety outcomes were pooled as risk ratios from event counts. The searches identified 1552 records. After deduplication, 379 records were screened and 85 full-text reports were assessed. Eleven reports were included in the systematic review. TETON-1 and TETON-2 were the two direct-IPF randomized trials within the coordinated TETON program eligible for primary meta-analysis. Inhaled treprostinil improved absolute forced vital capacity (FVC) change at 52 weeks compared with placebo (MD 111.28 mL, 95% CI 77.61 to 144.95, p < 0.001; I squared 8.5%). Clinical worsening was reduced (HR 0.69, 95% CI 0.57 to 0.84, p < 0.001; I squared 0.0%). Acute exacerbation was lower in pooled analysis (HR 0.52, 95% CI 0.30 to 0.91, p = 0.022), while all-cause mortality did not differ significantly (HR 0.70, 95% CI 0.47 to 1.05, p = 0.086). Adverse-event discontinuation, cough and headache were more frequent with treprostinil. In direct IPF randomized evidence, inhaled treprostinil preserved lung function and reduced clinical worsening at 52 weeks.

PMID 42930051
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PubMedPulmonary circulation2026-10-25

Salvage Use of Sotatercept for Potts Shunt in Refractory Pulmonary Hypertension.

Xu Zhuoyuan Z, Zhang Mingjie M, Chen Yiwei Y, Yu Xiafeng X et al.

The reverse Potts shunt (RPS) is a promising palliative intervention for pediatric patients with drug-refractory severe pulmonary arterial hypertension (PAH), yet it carries substantial risks of perioperative mortality and hemodynamic instability. We report two high-risk pediatric female patients who developed acute hemodynamic instability and recurrent pulmonary hypertensive crises following successful percutaneous RPS creation. Despite maximal targeted triple therapy and vasoactive support, no sustained improvement was observed. Following multidisciplinary consensus, a single salvage dose of sotatercept (0.3 mg/kg) was administered. Both patients demonstrated rapid, hemodynamic stabilization within 48 h, enabling the swift withdrawal of vasoactive support and inhaled nitric oxide within 1 week. Follow-up echocardiography revealed significant recovery of right ventricular function, and both patients were successfully weaned off treprostinil. These findings suggest that short-term perioperative sotatercept can serve as an effective acute rescue therapy to safeguard high-risk surgical interventions in critical pediatric PAH.

PMID 42924047
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PubMedTherapeutic advances in respiratory disease2026-10-25

Therapeutic advances in pulmonary fibrosis.

Temizel Ekrem E, Franco Amaral Alexandre A, Kaenmuang Punchalee P, Logan Niamh N et al.

Pharmacological treatment of idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) is evolving from two established antifibrotic agents towards a broader pipeline of therapies. Current standard treatment with nintedanib and pirfenidone slows the decline in forced vital capacity, but does not halt disease progression, and long-term use may be limited by tolerability issues. This review summarises major profibrotic mechanisms relevant to drug development and discusses therapies in advanced or emerging clinical programmes, including the recently FDA-approved nerandomilast, positive results from the phase 3 trial of inhaled treprostinil, as well as promising mid-stage, pathway-directed agents targeting a broad range of profibrotic signalling pathways. We also discuss how negative or terminated programmes highlight recurring limitations related to efficacy, safety, trial duration, endpoint selection, and patient heterogeneity. Finally, we outline current shifts in trial design and outcome assessment, including increasing emphasis on patient-centred and composite endpoints alongside physiological measures, and discuss the rationale for add-on and combination approaches in future antifibrotic therapy for IPF and PPF.

PMID 42921845
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PubMedRespiratory investigation2026-10-23

Pulmonary diffusion capacity to select patients for echocardiographic evaluation of pulmonary hypertension in interstitial lung disease: Impact of interstitial lung disease phenotype.

Ito Noriaki N, Masuda Takeshi T, Shimoji Kiyofumi K, Yamaguchi Kakuhiro K et al.

Treprostinil inhalation for pulmonary hypertension (PH) with interstitial lung disease (ILD) has increased the importance of PH screening. We aimed to determine whether pulmonary function tests performed for ILD management could identify patients with possible PH on echocardiography, according to ILD subtype. In this cross-sectional study, patients with ILD who underwent pulmonary function tests and echocardiography within three months were enrolled consecutively. The correlation between pulmonary function test results and peak tricuspid regurgitation velocity (TRV) was assessed using Spearman's rank correlation coefficient. We defined possible PH as peak TRV > 2.8 m/s. The screening performance for possible PH was evaluated using ROC analysis. In total, 278 patients were analysed. Among pulmonary function test values, percent predicted diffusing capacity of the lung for carbon monoxide (%DLCO) most significantly correlated with TRV (ρ = -0.468, p < 0.001). In the ROC curve analysis for identifying possible PH, %DLCO exhibited the highest area under the curve (AUC) of 0.750. The AUC for combined pulmonary fibrosis and emphysema (CPFE), idiopathic interstitial pneumonias (IIPs), and collagen vascular disease-related interstitial pneumonia (CVD-IP) were 0.856, 0.747, and 0.671, respectively. Among patients with IIPs, the AUC was higher when Brinkman Index was ≥600 than when it was <600 (0.881 vs 0.620). Based on numerical trends, %DLCO showed potentially high screening performance in patients with CPFE or IIPs with a history of heavy smoking. Echocardiographic evaluation for possible PH may be warranted in this population with reduced %DLCO.

PMID 42913275
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PubMedLung2026-10-22

Phase 3 Antifibrotic-Directed Therapy Outcomes in Idiopathic Pulmonary Fibrosis: Beyond FVC Decline.

Kashizaki Fumihiro F, Watanabe Shohei S, Orii Ryusuke R, Lin Hanming H et al.

Phase 3 evidence for antifibrotic-directed therapies in idiopathic pulmonary fibrosis (IPF) has expanded, but direct head-to-head trials are lacking. Forced vital capacity (FVC) decline remains the principal trial outcome, although it may not fully capture clinically relevant differences among therapies. We systematically reviewed phase 3 randomized placebo-controlled trials of antifibrotic-directed therapies in IPF. FVC effects versus placebo were descriptively summarized using standardized 48-52-week estimates. All-cause treatment discontinuation and all-cause mortality were analyzed using placebo-controlled odds ratios. Sensitivity analyses assessed follow-up duration, background antifibrotic therapy, and trial-level consistency. Ten trials including 5,386 randomized participants were included. Pirfenidone and nintedanib showed consistent reductions in FVC decline versus placebo. Inhaled treprostinil showed a favorable FVC signal in the combined TETON-1/2 evidence, largely as add-on therapy with background antifibrotic treatment. Without background antifibrotic therapy, several therapies showed directionally favorable but imprecise FVC effects, without evidence of equivalence. Nintedanib and inhaled treprostinil were associated with higher odds of all-cause treatment discontinuation versus placebo: OR 1.39 (95% CI, 1.02-1.88) and OR 1.39 (95% CI, 1.08-1.78), respectively. Pirfenidone was the only therapy associated with lower odds of all-cause mortality (OR, 0.50; 95% CI, 0.31-0.83), based on published pooled ASCEND/CAPACITY mortality data and the Japanese phase 3 trial. FVC decline remains essential but may not fully distinguish antifibrotic-directed therapies in IPF. Integrating FVC effects, background therapy, treatment continuation, and mortality provides a cautious, context-dependent framework for interpreting expanding phase 3 evidence.

PMID 42907709
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PubMedRespiration; international review of thoracic diseases2026-10-18

Pulmonary hypertension associated with lung disease - an expert review from the Cologne Conference.

Ulrich Silvia S, Milger Katrin K, Halank Michael M, Klose Hans H et al.

Pulmonary hypertension associated with chronic lung disease (PH_lung) is a frequent complication of chronic obstructive pulmonary disease, interstitial lung disease, and other parenchymal lung disorders, and is associated with impaired exercise capacity, reduced quality of life and poor survival. Despite increasing recognition of its clinical importance, major challenges remain regarding early diagnosis, hemodynamic classification, and optimal treatment. Following the 7th World Symposium on Pulmonary Hypertension, a panel of experts reviewed from Germany and International collaborators summarized recent evidence and discussed unresolved issues relevant to everyday clinical practice. This review summarizes current concepts of the diagnosis and management of PH_lung, with particular emphasis on multimodal screening strategies integrating pulmonary function testing, biomarkers, echocardiography, cardiopulmonary exercise testing, and thoracic imaging to improve patient selection for right heart catheterization. We discuss practical aspects of hemodynamic assessment, overlapping phenotypes, including connective tissue disease, sarcoidosis, and the emerging pulmonary vascular "lung phenotype", and current evidence supporting targeted therapies with emphasis on patients' pathways in real-world settings. While inhaled treprostinil has become the first approved treatment for pulmonary hypertension associated with interstitial lung disease in several countries, no pulmonary hypertension-specific therapy is currently recommended routinely for COPD-associated PH, highlighting the need for careful patient phenotyping and individualized management in expert centres. The review further addresses indications for lung transplantation and outlines future research priorities. Earlier recognition, multidisciplinary collaboration, and continued translational and clinical research are essential to improve outcomes in this highly vulnerable patient population.

PMID 42891961
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