TY - JOUR T1 - 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 multicentre study JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.00124-2020 VL - 57 IS - 2 SP - 2000124 AU - Robert A. Lewis AU - Iain Armstrong AU - Carmel Bergbaum AU - Melanie J. Brewis AU - John Cannon AU - Athanasios Charalampopoulos AU - A. Colin Church AU - J. Gerry Coghlan AU - Rachel J. Davies AU - Konstantinos Dimopoulos AU - Charlie Elliot AU - J. Simon R. Gibbs AU - Wendy Gin-Sing AU - Gulam Haji AU - Abdul G. Hameed AU - Luke S. Howard AU - Martin K. Johnson AU - Aleksander Kempny AU - David G. Kiely AU - Francesco Lo Giudice AU - Colm McCabe AU - Andrew J. Peacock AU - Oyinkansola Peleyeju AU - Joanna Pepke-Zaba AU - Gary Polwarth AU - Laura Price AU - Ian Sabroe AU - Benjamin E. Schreiber AU - Karen Sheares AU - Dolores Taboada AU - A.A. Roger Thompson AU - Mark R. Toshner AU - Ivy Wanjiku AU - S. John Wort AU - Janelle Yorke AU - Robin Condliffe Y1 - 2021/02/01 UR - http://erj.ersjournals.com/content/57/2/2000124.abstract N2 - 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.The emPHasis-10 health-related quality of life score is an independent predictor of mortality in idiopathic and connective tissue disease-related pulmonary arterial hypertension, and has utility in risk stratification. https://bit.ly/2MrLFLn ER -