Shining a spotlight on pulmonary hypertension associated with interstitial lung disease care: The latest advances in diagnosis and treatment.

IF 2.3 4区 医学 Q2 HEALTH CARE SCIENCES & SERVICES
Steven D Nathan, Michael R Stinchon, Sara Atcheson, Laura Simone, Marykate Nelson
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Abstract

Pulmonary hypertension associated with interstitial lung disease (PH-ILD) is a complex condition in which 2 consequential diseases interact and increase negative outcomes. Although the pathophysiologic mechanisms of PH-ILD are not yet well understood, the pronounced effect on functional status, supplemental oxygen requirements, health care resource utilization, and mortality that frequently accompany this diagnosis are well documented. A critical feature that complicates pathophysiologic understanding of PH-ILD is that progression of the pulmonary vascular disease does not always appear to be driven by the underlying lung disease. As PH-ILD is a progressive disease, early recognition and treatment initiation have the potential to delay the increased burden it creates. Historically, therapeutic development within pulmonary hypertension has concentrated on pulmonary arterial hypertension (PAH). However, PH-ILD and PAH are categorically distinct-belonging to distinct PH groups owing to differing pathophysiological mechanisms and therapeutic implications. PAH and PH-ILD may have numerous similarities; however, when PAH therapies have been studied in patients with PH-ILD, inconclusive efficacy (bosentan, sildenafil, tadalafil, iloprost) and at times deleterious safety findings (riociguat, ambrisentan) have been observed. Despite the paucity of evidence to support PAH therapy use in this patient population, widespread off-label use of PAH therapies arose as a result of a historical lack of PH-ILD-approved treatment. Recently, inhaled treprostinil-a prostacyclin analog-has become the first therapy approved for treatment of PH-ILD. In the phase 3 INCREASE trial, inhaled treprostinil was effective in improving 6-minute walk distance (the primary endpoint; P < 0.001) as well as N-terminal pro-B-type natriuretic peptide levels (P < 0.001). The approval of inhaled treprostinil in 2022 facilitates evidence-based therapeutic management. In addition, the 7th World Symposium on Pulmonary Hypertension has recently published an extensive summary of clinical research to date in PH-ILD. The proceedings from the 7th World Symposium on Pulmonary Hypertension provide timely recommendations for investigation of PH-ILD and a framework for assessing treatment needs. The therapeutic landscape advances are poised to transform PH-ILD care and improve outcomes for patients with PH-ILD.

聚焦与间质性肺疾病相关的肺动脉高压:诊断和治疗的最新进展。
肺动脉高压伴间质性肺病(PH-ILD)是一种复杂的病症,其中两种后果性疾病相互作用,增加了不良后果。尽管人们对 PH-ILD 的病理生理学机制尚不十分清楚,但这种诊断对患者功能状态、补氧需求、医疗资源利用率和死亡率的显著影响却有据可查。使人们对 PH-ILD 的病理生理学认识复杂化的一个关键特征是,肺血管疾病的进展似乎并不总是由潜在的肺部疾病所驱动。由于 PH-ILD 是一种进展性疾病,早期识别和开始治疗有可能延缓其造成的负担增加。从历史上看,肺动脉高压(PAH)的治疗发展主要集中在肺动脉高压上。然而,PH-ILD 和 PAH 因其不同的病理生理机制和治疗意义而截然不同,属于不同的 PH 组别。PAH 和 PH-ILD 可能有许多相似之处;然而,在对 PH-ILD 患者进行 PAH 治疗研究时,却发现疗效不确定(波生坦、西地那非、他达拉非、伊洛前列素),有时还会出现有害的安全性结果(利奥吉曲特、安立生坦)。尽管支持在这一患者群体中使用 PAH 疗法的证据很少,但由于历史上缺乏 PH-ILD 批准的治疗方法,因此 PAH 疗法在标签外广泛使用。最近,吸入式曲普瑞替尼--一种前列环素类似物--成为首个获准用于治疗 PH-ILD 的疗法。在 3 期 INCREASE 试验中,吸入曲普瑞替尼可有效改善 6 分钟步行距离(主要终点;P < 0.001)和 N 端前 B 型钠尿肽水平(P < 0.001)。2022年吸入式曲普瑞替尼获批有助于循证治疗管理。此外,第七届世界肺动脉高压研讨会最近发表了迄今为止关于 PH-ILD 临床研究的广泛总结。第七届世界肺动脉高压研讨会论文集为 PH-ILD 的研究提供了及时的建议,并为评估治疗需求提供了框架。治疗领域的进步有望改变 PH-ILD 的治疗,改善 PH-ILD 患者的预后。
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来源期刊
Journal of managed care & specialty pharmacy
Journal of managed care & specialty pharmacy Health Professions-Pharmacy
CiteScore
3.50
自引率
4.80%
发文量
131
期刊介绍: JMCP welcomes research studies conducted outside of the United States that are relevant to our readership. Our audience is primarily concerned with designing policies of formulary coverage, health benefit design, and pharmaceutical programs that are based on evidence from large populations of people. Studies of pharmacist interventions conducted outside the United States that have already been extensively studied within the United States and studies of small sample sizes in non-managed care environments outside of the United States (e.g., hospitals or community pharmacies) are generally of low interest to our readership. However, studies of health outcomes and costs assessed in large populations that provide evidence for formulary coverage, health benefit design, and pharmaceutical programs are of high interest to JMCP’s readership.
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