强调-10健康相关生活质量评分预测特发性和结缔组织病相关肺动脉高压患者的预后:来自英国一项多中心研究的结果

Robert A. Lewis, I. Armstrong, Carmel Bergbaum, M. Brewis, J. Cannon, A. Charalampopoulos, A. C. Church, J. Coghlan, R. Davies, K. Dimopoulos, C. Elliot, J. Gibbs, W. Gin-Sing, Gulam S. Haji, A. Hameed, L. Howard, Martin Johnson, A. Kempny, D. Kiely, F. Lo Giudice, C. McCabe, Oyinkansola Peleyeju, J. Pepke-Zaba, Gary Polwarth, L. Price, I. Sabroe, B. Schreiber, K. Sheares, D. Taboada, A. Thompson, M. Toshner, Ivy Wanjiku, S. Wort, J. Yorke, R. Condliffe
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引用次数: 8

摘要

健康相关生活质量(HRQoL)评分评估肺动脉高压(PAH)的症状负担,但有关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测量来评估这些关系。1745名特发性或结缔组织病相关的PAH患者在2014- 2017年间在6个英国转诊中心完成了emPHasis-10问卷调查。评估与运动能力和WHO功能分级(FC)的相关性,并测试探索性风险分层阈值。强调-10评分与6分钟步行距离(r=0.546)、增加穿梭步行距离(r=-0.504)和WHO FC (r=0.497;P均<0.0001)。各WHO FC间的强调-10分布差异显著(p均<0.0001)。在多变量分析中,强调-10(而非WHO FC)是死亡率的独立预测因子。在风险分层方法中,0-16分、17-33分和34-50分分别确定了一年死亡率为5%、10%和23%的事件患者。WHO FC III患者的生存可以使用emPHasis-10评分≥34分进一步分层(p<0.01)。在随访中,改善了强调10的患者的运动能力得到了改善(p<0.0001),并且过渡到危险组的患者的生存率与最初处于这些危险组的患者相似。在特发性或结缔组织病相关PAH患者中,emPHasis-10评分是一个独立的预后指标。除了目前使用的参数外,它还可以用于风险分层。强调-10评分的提高与运动能力的提高有关。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
EmPHasis-10 health-related quality of life score predicts outcomes in patients with idiopathic and connective tissue disease-associated pulmonary arterial hypertension: results from a UK multi-centre study
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 or connective tissue disease-associated PAH who had completed emPHasis-10 questionnaires between 2014-17 at 6 UK referral centres were identified. Correlations with exercise capacity and WHO functional class (FC) were assessed, and exploratory risk stratification thresholds were tested. Moderate correlations were seen between emPHasis-10 scores and 6-minute walk distance (r=0.546), incremental shuttle walking distance (r=-0.504) and WHO FC (r=0.497; p all <0.0001). Distribution of emPHasis-10 differed significantly between each WHO FC (p all <0.0001). At multivariate analysis, emPHasis-10, but not WHO FC, was an independent predictor of mortality. In a risk stratification approach, scores of 0-16, 17-33 and 34-50 identified incident patients with oneyear mortality of 5%, 10% and 23%, respectively. Survival of patients in WHO FC III could be further stratified using an emPHasis-10 score ≥34 (p<0.01). At follow-up, patients with improved emPHasis10 had improved exercise capacity (p<0.0001), and patients who transitioned risk groups demonstrated similar survival to patients originally in those risk groups. The emPHasis-10 score is an independent prognostic marker in patients with idiopathic or connective tissue disease-associated PAH. It has utility in risk stratification in addition to currently used parameters. Improvement in emPHasis-10 score is associated with improved exercise capacity.
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