Age- and Sex-Related Differences in Patients With Wild-Type Transthyretin Amyloidosis : Insights From THAOS

Background: Wild-type transthyretin amyloidosis (ATTRwt amyloidosis) is primarily diagnosed in elderly men but diagnoses in younger patients and women have recently increased. Objectives: The purpose of this study was to examine age- and sex-related differences in patients with ATTRwt amyloidosis en...

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Detalles Bibliográficos
Autor: the THAOS Investigators
Tipo de recurso: artículo
Fecha de publicación:2024
País:España
Institución:Universidad Francisco de Vitoria
Repositorio:DDFV. Repositorio Institucional de la Universidad Francisco de Vitoria
Idioma:inglés
OAI Identifier:oai:ddfv.ufv.es:10641/7361
Acceso en línea:https://hdl.handle.net/10641/7361
Access Level:acceso abierto
Palabra clave:age
amyloid
cardiomyopathy
sex differences
Cardiology and Cardiovascular Medicine
Journal Article
Yes
yes
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spelling Age- and Sex-Related Differences in Patients With Wild-Type Transthyretin Amyloidosis : Insights From THAOSthe THAOS Investigatorsageamyloidcardiomyopathysex differencesCardiology and Cardiovascular MedicineJournal ArticleYesyesBackground: Wild-type transthyretin amyloidosis (ATTRwt amyloidosis) is primarily diagnosed in elderly men but diagnoses in younger patients and women have recently increased. Objectives: The purpose of this study was to examine age- and sex-related differences in patients with ATTRwt amyloidosis enrolled in the THAOS (Transthyretin Amyloidosis Outcomes Survey). Methods: THAOS was a global, longitudinal, observational survey of patients with transthyretin amyloidosis, including both hereditary and wild-type disease, and asymptomatic carriers of pathogenic transthyretin gene variants. Patient characteristics at enrollment were analyzed by age at enrollment and sex (data cutoff date: August 1, 2022). Results: Of 1,251 patients with ATTRwt amyloidosis, 13.7%, 49.1%, 34.5%, and 2.8% were aged <70 years, 70 to 79 years, 80 to 89 years, and ≥90 years, respectively. The proportion of women increased with age, from 4.1% in patients aged <70 years to 14.3% in patients aged ≥90 years. In the respective age groups, median time from symptom onset to diagnosis overall (male, female) was 1.7 (1.3, 5.2), 2.0 (2.0, 2.2), 1.8 (1.9, 0.8), and 0.7 (0.6, 2.5) years. A Karnofsky Performance Status score ≤70 was observed in 17.1%, 30.1%, 46.1%, and 44.4% of patients aged <70 years, 70 to 79 years, 80 to 89 years, and ≥90 years, respectively. Conclusions: In this THAOS analysis of patients with ATTRwt amyloidosis, patients were diagnosed an average of 2 years after symptom onset, with the greatest diagnostic delay in women aged <70 years at 5 years. Patients were predominantly men, but the proportion of women increased with age. A substantial proportion of patients had significant functional impairment regardless of age.Facultad de Medicina20242024-08-0120242024-08-01journal articlehttp://purl.org/coar/resource_type/c_6501info:eu-repo/semantics/articleapplication/pdfhttps://hdl.handle.net/10641/7361reponame:DDFV. Repositorio Institucional de la Universidad Francisco de Vitoriainstname:Universidad Francisco de VitoriaInglésengopen accesshttp://purl.org/coar/access_right/c_abf2http://creativecommons.org/licenses/by-nc-nd/4.0/info:eu-repo/semantics/openAccessoai:ddfv.ufv.es:10641/73612026-06-11T12:44:57Z
dc.title.none.fl_str_mv Age- and Sex-Related Differences in Patients With Wild-Type Transthyretin Amyloidosis : Insights From THAOS
title Age- and Sex-Related Differences in Patients With Wild-Type Transthyretin Amyloidosis : Insights From THAOS
spellingShingle Age- and Sex-Related Differences in Patients With Wild-Type Transthyretin Amyloidosis : Insights From THAOS
the THAOS Investigators
age
amyloid
cardiomyopathy
sex differences
Cardiology and Cardiovascular Medicine
Journal Article
Yes
yes
title_short Age- and Sex-Related Differences in Patients With Wild-Type Transthyretin Amyloidosis : Insights From THAOS
title_full Age- and Sex-Related Differences in Patients With Wild-Type Transthyretin Amyloidosis : Insights From THAOS
title_fullStr Age- and Sex-Related Differences in Patients With Wild-Type Transthyretin Amyloidosis : Insights From THAOS
title_full_unstemmed Age- and Sex-Related Differences in Patients With Wild-Type Transthyretin Amyloidosis : Insights From THAOS
title_sort Age- and Sex-Related Differences in Patients With Wild-Type Transthyretin Amyloidosis : Insights From THAOS
dc.creator.none.fl_str_mv the THAOS Investigators
author the THAOS Investigators
author_facet the THAOS Investigators
author_role author
dc.contributor.none.fl_str_mv Facultad de Medicina
dc.subject.none.fl_str_mv age
amyloid
cardiomyopathy
sex differences
Cardiology and Cardiovascular Medicine
Journal Article
Yes
yes
topic age
amyloid
cardiomyopathy
sex differences
Cardiology and Cardiovascular Medicine
Journal Article
Yes
yes
description Background: Wild-type transthyretin amyloidosis (ATTRwt amyloidosis) is primarily diagnosed in elderly men but diagnoses in younger patients and women have recently increased. Objectives: The purpose of this study was to examine age- and sex-related differences in patients with ATTRwt amyloidosis enrolled in the THAOS (Transthyretin Amyloidosis Outcomes Survey). Methods: THAOS was a global, longitudinal, observational survey of patients with transthyretin amyloidosis, including both hereditary and wild-type disease, and asymptomatic carriers of pathogenic transthyretin gene variants. Patient characteristics at enrollment were analyzed by age at enrollment and sex (data cutoff date: August 1, 2022). Results: Of 1,251 patients with ATTRwt amyloidosis, 13.7%, 49.1%, 34.5%, and 2.8% were aged <70 years, 70 to 79 years, 80 to 89 years, and ≥90 years, respectively. The proportion of women increased with age, from 4.1% in patients aged <70 years to 14.3% in patients aged ≥90 years. In the respective age groups, median time from symptom onset to diagnosis overall (male, female) was 1.7 (1.3, 5.2), 2.0 (2.0, 2.2), 1.8 (1.9, 0.8), and 0.7 (0.6, 2.5) years. A Karnofsky Performance Status score ≤70 was observed in 17.1%, 30.1%, 46.1%, and 44.4% of patients aged <70 years, 70 to 79 years, 80 to 89 years, and ≥90 years, respectively. Conclusions: In this THAOS analysis of patients with ATTRwt amyloidosis, patients were diagnosed an average of 2 years after symptom onset, with the greatest diagnostic delay in women aged <70 years at 5 years. Patients were predominantly men, but the proportion of women increased with age. A substantial proportion of patients had significant functional impairment regardless of age.
publishDate 2024
dc.date.none.fl_str_mv 2024
2024-08-01
2024
2024-08-01
dc.type.none.fl_str_mv journal article
http://purl.org/coar/resource_type/c_6501
dc.type.openaire.fl_str_mv info:eu-repo/semantics/article
format article
dc.identifier.none.fl_str_mv https://hdl.handle.net/10641/7361
url https://hdl.handle.net/10641/7361
dc.language.none.fl_str_mv Inglés
eng
language_invalid_str_mv Inglés
language eng
dc.rights.none.fl_str_mv open access
http://purl.org/coar/access_right/c_abf2

http://creativecommons.org/licenses/by-nc-nd/4.0/
dc.rights.openaire.fl_str_mv info:eu-repo/semantics/openAccess
rights_invalid_str_mv open access
http://purl.org/coar/access_right/c_abf2

http://creativecommons.org/licenses/by-nc-nd/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.source.none.fl_str_mv reponame:DDFV. Repositorio Institucional de la Universidad Francisco de Vitoria
instname:Universidad Francisco de Vitoria
instname_str Universidad Francisco de Vitoria
reponame_str DDFV. Repositorio Institucional de la Universidad Francisco de Vitoria
collection DDFV. Repositorio Institucional de la Universidad Francisco de Vitoria
repository.name.fl_str_mv
repository.mail.fl_str_mv
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