RT Journal Article
SR Electronic
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
FD European Respiratory Society
SP 2000124
DO 10.1183/13993003.00124-2020
VO 57
IS 2
A1 Robert A. Lewis
A1 Iain Armstrong
A1 Carmel Bergbaum
A1 Melanie J. Brewis
A1 John Cannon
A1 Athanasios Charalampopoulos
A1 A. Colin Church
A1 J. Gerry Coghlan
A1 Rachel J. Davies
A1 Konstantinos Dimopoulos
A1 Charlie Elliot
A1 J. Simon R. Gibbs
A1 Wendy Gin-Sing
A1 Gulam Haji
A1 Abdul G. Hameed
A1 Luke S. Howard
A1 Martin K. Johnson
A1 Aleksander Kempny
A1 David G. Kiely
A1 Francesco Lo Giudice
A1 Colm McCabe
A1 Andrew J. Peacock
A1 Oyinkansola Peleyeju
A1 Joanna Pepke-Zaba
A1 Gary Polwarth
A1 Laura Price
A1 Ian Sabroe
A1 Benjamin E. Schreiber
A1 Karen Sheares
A1 Dolores Taboada
A1 A.A. Roger Thompson
A1 Mark R. Toshner
A1 Ivy Wanjiku
A1 S. John Wort
A1 Janelle Yorke
A1 Robin Condliffe
YR 2021
UL http://erj.ersjournals.com/content/57/2/2000124.abstract
AB 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