Pulmonary hypertension (PH) is a feared complication in patients with any chronic lung disease,1 but those with lung fibrosis have an especially poor mortality and the morbidity burden is very high.2 3 Up to very recently, there has been an almost nihilistic attitude towards these patients, since the lack of treatment options restricted the indication of a definitive diagnosis through right heart catheterisation (RHC) to lung transplant evaluation and enrolment in a clinical trial.4 Although with sound rationale, randomised placebo-controlled trials (RCT) with pulmonary vasodilators, have been negative or at worse detrimental5 (figure 1). That was until recently. The INCREASE study6 was the first double-blind RCT that had a positive outcome with respect to primary outcome (6-minute walking distance) as well as an improvement in brain natriuretic peptide levels and reduced clinical worsening events. Patients, with a wide range of underlying interstitial lung diseases (ILDs) and RHC-diagnosed PH, received 16 weeks of inhaled treprostinil up to nine doses a day compared with placebo. Furthermore, in an open-label extension (OLE) study, benefits were seen up to 52 weeks including a reduction in ILD exacerbations.7 Other post hoc studies have shown a dose dependent improvement8 and that patients receiving treprostinil are less likely to experience further disease progression events after an initial event.9 Figure 1 Chronology of randomised, double-blind, placebo-controlled trials with pulmonary …

中文翻译：

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吸入曲前列环素用于间质性肺疾病和肺动脉高压患者：大胆地走向其他肺血管扩张剂未曾涉足的领域？

肺动脉高压 (PH) 是任何慢性肺部疾病患者都担心的并发症，1 但肺纤维化患者的死亡率特别低，发病率负担非常高。2 3 直到最近，人们还抱有一种近乎虚无主义的态度对于这些患者，因为缺乏治疗选择限制了通过右心导管插入术 (RHC) 进行肺移植评估和参加临床试验的明确诊断的指征。 4 尽管有合理的理由，但随机安慰剂对照试验 (RCT)肺血管扩张剂的结果为阴性或更严重的是有害5（图1）。那是直到最近。INCREASE 研究 6 是第一个双盲随机对照试验，在主要结局（6 分钟步行距离）方面取得了积极成果，并且改善了脑钠尿肽水平并减少了临床恶化事件。患有多种潜在间质性肺疾病 (ILD) 和 RHC 诊断的 PH 的患者与安慰剂相比，接受了为期 16 周的吸入曲前列环素（每天最多 9 剂）治疗。此外，在一项开放标签延伸 (OLE) 研究中，获益长达 52 周，包括 ILD 恶化的减少。7 其他事后研究显示出剂量依赖性改善8，并且接受曲前列环素的患者不太可能出现进一步的疾病初始事件后的进展事件。9 图 1 肺部随机、双盲、安慰剂对照试验的年表……