Rahaghi Franck F, Kolaitis Nicholas A, Adegunsoye Ayodeji, de Andrade Joao A, Flaherty Kevin R, Lancaster Lisa H, Lee Joyce S, Levine Deborah J, Preston Ioana R, Safdar Zeenat, Saggar Rajan, Sahay Sandeep, Scholand Mary Beth, Shlobin Oksana A, Zisman David A, Nathan Steven D
Advanced Lung Disease Clinic, Cleveland Clinic Florida, Weston, FL.
UCSF Pulmonary and Critical Care, San Francisco, CA.
Chest. 2022 Jul;162(1):145-155. doi: 10.1016/j.chest.2022.02.012. Epub 2022 Feb 15.
Pulmonary hypertension (PH) is a common complication of interstitial lung disease (ILD) and is associated with worse outcomes and increased mortality. Evaluation of PH is recommended in lung transplant candidates, but there are currently no standardized screening approaches. Trials have identified therapies that are effective in this setting, providing another rationale to routinely screen patients with ILD for PH.
What screening strategies for identifying PH in patients with ILD are supported by expert consensus?
The study convened a panel of 16 pulmonologists with expertise in PH and ILD, and used a modified Delphi consensus process with three surveys to identify PH screening strategies. Survey 1 consisted primarily of open-ended questions. Surveys 2 and 3 were developed from responses to survey 1 and contained statements about PH screening that panelists rated from -5 (strongly disagree) to 5 (strongly agree).
Panelists reached consensus on several triggers for suspicion of PH including the following: symptoms, clinical signs, findings on chest CT scan or other imaging, abnormalities in pulse oximetry, elevations in brain natriuretic peptide (BNP) or N-terminal pro-brain natriuretic peptide (NT-proBNP), and unexplained worsening in pulmonary function tests or 6-min walk distance. Echocardiography and BNP/NT-proBNP were identified as screening tools for PH. Right heart catheterization was deemed essential for confirming PH.
Many patients with ILD may benefit from early evaluation of PH now that an approved therapy is available. Protocols to evaluate patients with ILD often overlap with evaluations for pulmonary hypertension-interstitial lung disease and can be used to assess the risk of PH. Because standardized approaches are lacking, this consensus statement is intended to aid physicians in the identification of patients with ILD and possible PH, and provide guidance for timely right heart catheterization.
肺动脉高压（PH）是间质性肺疾病（ILD）的常见并发症，与更差的预后和更高的死亡率相关。建议对肺移植候选者进行PH评估，但目前尚无标准化的筛查方法。试验已确定了在此情况下有效的治疗方法，这为常规筛查ILD患者是否患有PH提供了另一个理由。
专家共识支持哪些用于识别ILD患者中PH的筛查策略？
该研究召集了16名在PH和ILD方面具有专业知识的肺科医生组成的小组，并采用了经过修改的德尔菲共识过程，通过三轮调查来确定PH筛查策略。第一轮调查主要由开放式问题组成。第二轮和第三轮调查是根据对第一轮调查的回复制定的，包含关于PH筛查的陈述，小组成员对这些陈述从-5（强烈反对）到5（强烈同意）进行评分。
小组成员就怀疑PH的几个触发因素达成了共识，包括以下方面：症状、临床体征、胸部CT扫描或其他影像学检查结果、脉搏血氧饱和度异常、脑钠肽（BNP）或N末端脑钠肽前体（NT-proBNP）升高，以及肺功能测试或6分钟步行距离无法解释的恶化。超声心动图和BNP/NT-proBNP被确定为PH的筛查工具。右心导管检查被认为是确诊PH必不可少的。
鉴于已有获批的治疗方法，许多ILD患者可能会从早期PH评估中受益。评估ILD患者的方案通常与评估肺动脉高压-间质性肺疾病重叠，可用于评估PH风险。由于缺乏标准化方法，本共识声明旨在帮助医生识别ILD和可能患有PH的患者，并为及时进行右心导管检查提供指导。
