Lewis, R.A., Armstrong, I., Bergbaum, C. et al. (33 more authors) (2021) 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. European Respiratory Journal, 57 (2). 2000124. ISSN 0903-1936
Abstract
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 one-year 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 emPHasis-10 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.
Metadata
Item Type: | Article |
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Authors/Creators: |
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Copyright, Publisher and Additional Information: | © 2021 ERS. This article is open access and distributed under the terms of the Creative Commons Attribution Licence 4.0 (http://creativecommons.org/licenses/by/4.0). |
Keywords: | Pulmonary Arterial Hypertension; Health Related Quality of Life; Patient Reported Outcomes Measure; emPHasis-10; Risk Stratification |
Dates: |
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Institution: | The University of Sheffield |
Academic Units: | The University of Sheffield > Sheffield Teaching Hospitals |
Funding Information: | Funder Grant number BRITISH HEART FOUNDATION FS/18/13/33281 |
Depositing User: | Symplectic Sheffield |
Date Deposited: | 13 Jul 2020 10:52 |
Last Modified: | 20 Jan 2022 11:48 |
Status: | Published |
Publisher: | European Respiratory Society (ERS) |
Refereed: | Yes |
Identification Number: | 10.1183/13993003.00124-2020 |
Related URLs: | |
Open Archives Initiative ID (OAI ID): | oai:eprints.whiterose.ac.uk:163153 |