Abstract
Introduction While the performance of the emPHasis-10 (e10) score has been evaluated against limited patient characteristics within the United Kingdom, there is an unmet need for exploring the performance of the e10 score among PAH patients in the United States.
Methods Using the Pulmonary Hypertension Association Registry, we evaluated relationships between the e10 score and demographic, functional, hemodynamics, and additional clinical characteristics at baseline and over time. Furthermore, we derived a minimally important difference (MID) estimate for the e10 score.
Results We analysed data from 565 PAH (75% female) adults 55.6±16.0 years of age. At baseline, the e10 score had notable correlation with factors expected to impact quality of life in the general population, including age, education level, income, smoking status, and body mass index. Clinically important parameters including six-minute walk distance and B-type natriuretic peptide/N-terminal-pro BNP were also significantly associated with e10 score at baseline and over time. We generated a MID estimate for the e10 score of −6.0 points (range −5.0 to −7.6 points).
Conclusions The e10 score was associated with demographic and clinical patient characteristics suggesting that HRQoL in PAH is influenced by both social factors and indicators of disease severity. Future studies are needed to demonstrate the impact of the e10 score on clinical decision-making and its potential utility for assessing clinically important interventions.
Footnotes
This manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.
Conflict of interest: Ms. Borgese has nothing to disclose.
Conflict of interest: Dr. Badesch reports grants, personal fees and other (long-term stockholder in Johnson & Johnson) from Actelion/Janssen/Johnson & Johnson, grants and personal fees from Arena/United Therapeutics/Lung LLC, grants and personal fees from Acceleron, grants and personal fees from Complexa, personal fees from Pfizer, grants and personal fees from Liquidia, grants from Altavant, grants and personal fees from Belleraphon, outside the submitted work.
Conflict of interest: Dr. Bull reports grants from Bayer, personal fees from Liquidia, outside the submitted work; and Steering committee for Liquidia (clinical trial)
Conflict of interest: Dr. Chakinala reports grants and other from Actelion, grants from Medtronic, grants and other from Reata Pharmaceuticals, grants from Eiger Bio Pharmaceuticals, grants from GeNO LLC, grants from GSK, grants and other from United Therapeutics, other from WedMD LLC (Medscape), other from SteadyMed Therapeutics Inc, other from Express Scripts Holding Company, grants, personal fees and other from Bayer, grants from Liquidia, grants and other from Phase Bio, grants from Complexa, grants from Altavant, grants and other from Acceleron, grants from Trio Health Analytics, grants, personal fees and other from Gilead Sciences, outside the submitted work.
Conflict of interest: Dr. De Marco reports that she serves as a consultant to Actelion, United Therapeutics, Arena, SCOPE/BIAL. She serves as PI on a multicenter trial sponsored by Acceleron.
Conflict of interest: Dr. Feldman reports personal fees from United Therapeutics, personal fees from Actelion, personal fees from Acceleron, personal fees from Bayer, personal fees from Altavant, outside the submitted work.
Conflict of interest: Dr. Ford reports personal fees from United Therapeutics, grants from United Therapeutics, personal fees from Bayer, personal fees from Actelion/Jannsen, grants from Actelion/Jannsen, grants from Liquidia, outside the submitted work.
Conflict of interest: Dr. Grinnan reports grants from Johnson and Johnson, grants from Bayer, grants from United Therapeutics, outside the submitted work.
Conflict of interest: Dr. KLINGER has nothing to disclose.
Conflict of interest: Mrs. Bolivar has nothing to disclose.
Conflict of interest: Oksana A. Shlobin
Conflict of interest: Dr. Frantz has nothing to disclose.
Conflict of interest: Dr. Sager reports personal fees from Actelion, personal fees from Bayer, personal fees from United Therapeutics, personal fees from Reata, personal fees from PhaseBio, outside the submitted work.
Conflict of interest: Dr. Mathai reports personal fees from Actelion, personal fees from Arena, personal fees from Liquidia, personal fees from United Therapeutics, outside the submitted work; and Member, Rare Disease Advisory Panel for the Patient Centered Outcomes Research Institute.
Conflict of interest: Dr. Kawut reports grants from NIH, non-financial support from ATS, and grants from Actelion, United Therapeutics, Gilead, Lung Biotech, Bayer, and Mallinkrodt to the Perelman School of Medicine for CME courses. Dr. Kawut reports grants and non-financial support from Cardiovascular Medical Research and Education Fund and non-financial support from Pulmonary Hypertension Association. Dr. Kawut has served in an advisory capacity (for grant review and other purposes) for United Therapeutics, Akros Pharmaceuticals, Glaxo SmithKline, and Complexa, Inc. without financial support or in-kind benefits.
Conflict of interest: Dr. Leary has nothing to disclose.
Conflict of interest: Dr. GRAY has nothing to disclose.
Conflict of interest: Dr. Popat has nothing to disclose.
Conflict of interest: Dr. Zamanian reports grants from Actelion, grants from Tenax, grants from PHaware, personal fees from Morphogen-IX, personal fees from Vivus, grants from United Therapeutics, outside the submitted work; In addition, Dr. Zamanian has a patent FK506 for PH issued.
- Received February 26, 2020.
- Accepted November 1, 2020.
- Copyright ©ERS 2020
LIBRARY USERS
Log in through your institution
Purchase access
