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
T2 - Results from a UK multicentre study
AU - Lewis, Robert A.
AU - Armstrong, Iain
AU - Bergbaum, Carmel
AU - Brewis, Melanie J.
AU - Cannon, John
AU - Charalampopoulos, Athanasios
AU - Church, A. Colin
AU - Coghlan, J. Gerry
AU - Davies, Rachel J.
AU - Dimopoulos, Konstantinos
AU - Elliot, Charlie
AU - Gibbs, J. Simon R.
AU - Gin-Sing, Wendy
AU - Haji, Gulam
AU - Hameed, Abdul G.
AU - Howard, Luke S.
AU - Johnson, Martin K.
AU - Kempny, Aleksander
AU - Kiely, David G.
AU - Giudice, Francesco Lo
AU - McCabe, Colm
AU - Peacock, Andrew J.
AU - Peleyeju, Oyinkansola
AU - Pepke-Zaba, Joanna
AU - Polwarth, Gary
AU - Price, Laura
AU - Sabroe, Ian
AU - Schreiber, Benjamin E.
AU - Sheares, Karen
AU - Taboada, Dolores
AU - Thompson, A. A.Roger
AU - Toshner, Mark R.
AU - Wanjiku, Ivy
AU - Wort, S. John
AU - Yorke, Janelle
AU - Condliffe, Robin
N1 - Publisher Copyright:
Copyright © ERS 2021. This article is open access and distributed under the terms of the Creative Commons Attribution Licence 4.0.
PY - 2021/2
Y1 - 2021/2
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.
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.
UR - http://www.scopus.com/inward/record.url?scp=85101899329&partnerID=8YFLogxK
U2 - 10.1183/13993003.00124-2020
DO - 10.1183/13993003.00124-2020
M3 - Journal article
C2 - 32631835
AN - SCOPUS:85101899329
SN - 0903-1936
VL - 57
JO - European Respiratory Journal
JF - European Respiratory Journal
IS - 2
M1 - 00124-2020
ER -