Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK.
Dept of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK.
Eur Respir J. 2021 Feb 25;57(2). doi: 10.1183/13993003.00124-2020. Print 2021 Feb.
Health-related quality of life (HRQoL) scores assess symptom burden in pulmonary arterial hypertension (PAH) but data regarding their role in prognostication and risk stratification are limited. We assessed these relationships using the emPHasis-10 HRQoL measure.1745 patients with idiopathic PAH (IPAH), drug-induced PAH (DPAH), heritable PAH (HPAH) (collectively "(I/D/H)PAH"), or connective tissue disease-associated PAH (CTD-PAH), who had completed emPHasis-10 questionnaires at one of six UK referral centres between 2014 and 2017, were identified. Correlations with exercise capacity and World Health Organization (WHO) functional class were assessed, and exploratory risk stratification thresholds were tested.Moderate correlations were seen between emPHasis-10 scores and 6-min walk distance (r=-0.546), incremental shuttle walk distance (r=-0.504) and WHO functional class (r=0.497) (all p<0.0001). Distribution of emPHasis-10 score differed significantly between each WHO functional class (all p<0.0001). On multivariate analysis, emPHasis-10 score, but not WHO functional class, was an independent predictor of mortality. In a risk stratification approach, scores of 0-16, 17-33 and 34-50 identified incident patients with 1-year mortality of 5%, 10% and 23%, respectively. Survival of patients in WHO functional class III could be further stratified using an emPHasis-10 score ≥34 (p<0.01). At follow-up, patients with improved emPHasis-10 scores had improved exercise capacity (p<0.0001) and patients who transitioned between risk groups demonstrated similar survival to patients originally in those risk groups.The emPHasis-10 score is an independent prognostic marker in patients with (I/D/H)PAH or CTD-PAH. It has utility in risk stratification in addition to currently used parameters. Improvement in emPHasis-10 score is associated with improved exercise capacity.
健康相关生活质量（HRQoL）评分可评估肺动脉高压（PAH）患者的症状负担，但关于其在预后和风险分层中的作用的数据有限。我们使用 emPHasis-10 HRQoL 量表评估了这些关系。1745 名特发性 PAH（IPAH）、药物诱导的 PAH（DPAH）、遗传性 PAH（HPAH）（统称为“（I/D/H）PAH”）或结缔组织疾病相关 PAH（CTD-PAH）患者，他们在 2014 年至 2017 年间的六个英国转诊中心之一完成了 emPHasis-10 问卷，被识别出来。评估了与运动能力和世界卫生组织（WHO）功能分类的相关性，并测试了探索性风险分层阈值。emPHasis-10 评分与 6 分钟步行距离（r=-0.546）、递增穿梭步行距离（r=-0.504）和 WHO 功能分类（r=0.497）之间存在中度相关性（均 p<0.0001）。emPHasis-10 评分的分布在每个 WHO 功能分类之间差异显著（均 p<0.0001）。多变量分析显示，emPHasis-10 评分而不是 WHO 功能分类是死亡率的独立预测因素。在风险分层方法中，评分 0-16、17-33 和 34-50 分别确定了 1 年死亡率为 5%、10%和 23%的患者。使用 emPHasis-10 评分≥34 可进一步分层 WHO 功能分类 III 患者（p<0.01）。在随访时，emPHasis-10 评分改善的患者运动能力改善（p<0.0001），而在风险组之间转换的患者与最初处于这些风险组的患者的生存情况相似。emPHasis-10 评分是（I/D/H）PAH 或 CTD-PAH 患者的独立预后标志物。它除了目前使用的参数外，在风险分层方面也有应用。emPHasis-10 评分的改善与运动能力的改善相关。
