Unmet needs in hereditary angioedema: an international survey of physicians

BackgroundHereditary angioedema (HAE) is a rare and potentially life-threatening genetic disorder characterized by unpredictable attacks of angioedema. MENTALIST (UnMEt Needs in herediTAry angioedema-a gLobal physIcian perSpecTive) is the first international survey uncovering unmet needs and identif...

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Autores: Buttgereit, Thomas, Aulenbacher, Felix, Adatia, Adil, Vera Ayala, Carolina, Ali Al-Nesf, Maryam, Altrichter, Sabine, Abuzakouk, Mohamed, Al-Ahmad, Mona, Mohammed Ali, Ramzy, Berardi, Alejandro, Boccon-Gibod, Isabelle, Bouillet, Laurence, Brussino, Luisa, Barešić, Marko, Busse, Paula J., Betschel, Stephen D., Chong Neto, Herberto, Calderón Llosa, Óscar, Craig, Timothy J., Dorr, Anthony D., Duarte Dortas, Sérgio, Fomina, Daria, Farkas, Henriette, Shen Fok, Jie, Grumach, Anete S., Greve, Jens, Guilarte, Mar, Gonçalo, Margarida, Grivcheva Panovska, Vesna, Hide, Michihiro, Hakl, Roman, Jindal, Ankur, Katelaris, Constance H., Kamaleswaran, Shailajah, Kinaciyan, Tamar, Latysheva, Elena, Larco Sousa, José Ignacio, Lleonart Bellfill, Ramon, Mobayed, Hassan, Metz, Martin, Nasr, Iman, Mitrevska, Natasa T., Nicola, Stefania, Salvador Parisi, Claudio Alberto, Porebski, Grzegorz, Peter, Jonny, Ferriani, Mariana Paes Leme, Rosario, Nelson, Şekerel, Bülent Enis, Sarquis Serpa, Faradiba, Stobiecki, Marcin, Trainotti, Susanne, Valerieva, Anna, Wongsa, Chamard, Wong, Jane C. Y., Yucel, Esra, Li, Yinglei, Nenci, Chiara, Maurer, Marcus, Magerl, Markus, Li, Philip H.
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2025
País:España
Institución:Universidad de Barcelona
Repositorio:Dipòsit Digital de la UB
OAI Identifier:oai:diposit.ub.edu:2445/222842
Acceso en línea:https://hdl.handle.net/2445/222842
Access Level:acceso abierto
Palabra clave:Edema angioneuròtic
Anafilaxi
Angioneurotic edema
Anaphylaxis
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spelling Unmet needs in hereditary angioedema: an international survey of physiciansButtgereit, ThomasAulenbacher, FelixAdatia, AdilVera Ayala, CarolinaAli Al-Nesf, MaryamAltrichter, SabineAbuzakouk, MohamedAl-Ahmad, MonaMohammed Ali, RamzyBerardi, AlejandroBoccon-Gibod, IsabelleBouillet, LaurenceBrussino, LuisaBarešić, MarkoBusse, Paula J.Betschel, Stephen D.Chong Neto, HerbertoCalderón Llosa, ÓscarCraig, Timothy J.Dorr, Anthony D.Duarte Dortas, SérgioFomina, DariaFarkas, HenrietteShen Fok, JieGrumach, Anete S.Greve, JensGuilarte, MarGonçalo, MargaridaGrivcheva Panovska, VesnaHide, MichihiroHakl, RomanJindal, AnkurKatelaris, Constance H.Kamaleswaran, ShailajahKinaciyan, TamarLatysheva, ElenaLarco Sousa, José IgnacioLleonart Bellfill, RamonMobayed, HassanMetz, MartinNasr, ImanMitrevska, Natasa T.Nicola, StefaniaSalvador Parisi, Claudio AlbertoPorebski, GrzegorzPeter, JonnyFerriani, Mariana Paes LemeRosario, NelsonŞekerel, Bülent EnisSarquis Serpa, FaradibaStobiecki, MarcinTrainotti, SusanneValerieva, AnnaWongsa, ChamardWong, Jane C. Y.Yucel, EsraLi, YingleiNenci, ChiaraMaurer, MarcusMagerl, MarkusLi, Philip H.Edema angioneuròticAnafilaxiAngioneurotic edemaAnaphylaxisBackgroundHereditary angioedema (HAE) is a rare and potentially life-threatening genetic disorder characterized by unpredictable attacks of angioedema. MENTALIST (UnMEt Needs in herediTAry angioedema-a gLobal physIcian perSpecTive) is the first international survey uncovering unmet needs and identifying barriers to optimal management in HAE following the latest update of the World Allergy Organization (WAO)/European Academy of Allergy and Clinical Immunology (EAACI) HAE guidelines.MethodsThis web-based survey comprised 24 questions on HAE management and unmet needs. HAE-expert physicians from the Angioedema Centers of Reference and Excellence network ranked unmet needs according to their own perspectives and their patients' perspectives, using a 10-point Likert scale ranging from 0 (not a challenge/unmet need at all) to 10 (huge challenge/unmet need).ResultsOf 64 respondents from 32 countries, most (91%) had > 5 years of experience in managing HAE. Overall, 48% of respondents (n = 31/64) reported that < 50% of their patients had achieved the WAO/EAACI HAE treatment goals of total disease control and normalization of life at the time of the survey. Implementation of consensus recommendations was found to be inconsistent across regions. Gaps in non-HAE-expert physician knowledge, treatment costs, and reimbursement for long-term prophylaxis were the highest-priority challenges according to the respondents. Burden of disease remains a challenge among patients, as reported by their physicians.ConclusionsThe MENTALIST findings highlight a need for removal of barriers to HAE treatment goals and propose a call to action to improve access to treatments, for greater provision of education for physicians and patients, critical collaboration with patient organizations and industry stakeholders and ultimately to optimize HAE care.Springer Science and Business Media LLC2025info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://hdl.handle.net/2445/222842Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))reponame:Dipòsit Digital de la UBinstname:Universidad de BarcelonaInglésReproducció del document publicat a: https://doi.org/10.1186/s13023-025-03739-8Orphanet Journal of Rare Diseases, 2025, vol. 20, num. 1https://doi.org/10.1186/s13023-025-03739-8cc by (c) Buttgereit, Thomas et al, 2025http://creativecommons.org/licenses/by/3.0/es/info:eu-repo/semantics/openAccessoai:diposit.ub.edu:2445/2228422026-05-27T06:46:51Z
dc.title.none.fl_str_mv Unmet needs in hereditary angioedema: an international survey of physicians
title Unmet needs in hereditary angioedema: an international survey of physicians
spellingShingle Unmet needs in hereditary angioedema: an international survey of physicians
Buttgereit, Thomas
Edema angioneuròtic
Anafilaxi
Angioneurotic edema
Anaphylaxis
title_short Unmet needs in hereditary angioedema: an international survey of physicians
title_full Unmet needs in hereditary angioedema: an international survey of physicians
title_fullStr Unmet needs in hereditary angioedema: an international survey of physicians
title_full_unstemmed Unmet needs in hereditary angioedema: an international survey of physicians
title_sort Unmet needs in hereditary angioedema: an international survey of physicians
dc.creator.none.fl_str_mv Buttgereit, Thomas
Aulenbacher, Felix
Adatia, Adil
Vera Ayala, Carolina
Ali Al-Nesf, Maryam
Altrichter, Sabine
Abuzakouk, Mohamed
Al-Ahmad, Mona
Mohammed Ali, Ramzy
Berardi, Alejandro
Boccon-Gibod, Isabelle
Bouillet, Laurence
Brussino, Luisa
Barešić, Marko
Busse, Paula J.
Betschel, Stephen D.
Chong Neto, Herberto
Calderón Llosa, Óscar
Craig, Timothy J.
Dorr, Anthony D.
Duarte Dortas, Sérgio
Fomina, Daria
Farkas, Henriette
Shen Fok, Jie
Grumach, Anete S.
Greve, Jens
Guilarte, Mar
Gonçalo, Margarida
Grivcheva Panovska, Vesna
Hide, Michihiro
Hakl, Roman
Jindal, Ankur
Katelaris, Constance H.
Kamaleswaran, Shailajah
Kinaciyan, Tamar
Latysheva, Elena
Larco Sousa, José Ignacio
Lleonart Bellfill, Ramon
Mobayed, Hassan
Metz, Martin
Nasr, Iman
Mitrevska, Natasa T.
Nicola, Stefania
Salvador Parisi, Claudio Alberto
Porebski, Grzegorz
Peter, Jonny
Ferriani, Mariana Paes Leme
Rosario, Nelson
Şekerel, Bülent Enis
Sarquis Serpa, Faradiba
Stobiecki, Marcin
Trainotti, Susanne
Valerieva, Anna
Wongsa, Chamard
Wong, Jane C. Y.
Yucel, Esra
Li, Yinglei
Nenci, Chiara
Maurer, Marcus
Magerl, Markus
Li, Philip H.
author Buttgereit, Thomas
author_facet Buttgereit, Thomas
Aulenbacher, Felix
Adatia, Adil
Vera Ayala, Carolina
Ali Al-Nesf, Maryam
Altrichter, Sabine
Abuzakouk, Mohamed
Al-Ahmad, Mona
Mohammed Ali, Ramzy
Berardi, Alejandro
Boccon-Gibod, Isabelle
Bouillet, Laurence
Brussino, Luisa
Barešić, Marko
Busse, Paula J.
Betschel, Stephen D.
Chong Neto, Herberto
Calderón Llosa, Óscar
Craig, Timothy J.
Dorr, Anthony D.
Duarte Dortas, Sérgio
Fomina, Daria
Farkas, Henriette
Shen Fok, Jie
Grumach, Anete S.
Greve, Jens
Guilarte, Mar
Gonçalo, Margarida
Grivcheva Panovska, Vesna
Hide, Michihiro
Hakl, Roman
Jindal, Ankur
Katelaris, Constance H.
Kamaleswaran, Shailajah
Kinaciyan, Tamar
Latysheva, Elena
Larco Sousa, José Ignacio
Lleonart Bellfill, Ramon
Mobayed, Hassan
Metz, Martin
Nasr, Iman
Mitrevska, Natasa T.
Nicola, Stefania
Salvador Parisi, Claudio Alberto
Porebski, Grzegorz
Peter, Jonny
Ferriani, Mariana Paes Leme
Rosario, Nelson
Şekerel, Bülent Enis
Sarquis Serpa, Faradiba
Stobiecki, Marcin
Trainotti, Susanne
Valerieva, Anna
Wongsa, Chamard
Wong, Jane C. Y.
Yucel, Esra
Li, Yinglei
Nenci, Chiara
Maurer, Marcus
Magerl, Markus
Li, Philip H.
author_role author
author2 Aulenbacher, Felix
Adatia, Adil
Vera Ayala, Carolina
Ali Al-Nesf, Maryam
Altrichter, Sabine
Abuzakouk, Mohamed
Al-Ahmad, Mona
Mohammed Ali, Ramzy
Berardi, Alejandro
Boccon-Gibod, Isabelle
Bouillet, Laurence
Brussino, Luisa
Barešić, Marko
Busse, Paula J.
Betschel, Stephen D.
Chong Neto, Herberto
Calderón Llosa, Óscar
Craig, Timothy J.
Dorr, Anthony D.
Duarte Dortas, Sérgio
Fomina, Daria
Farkas, Henriette
Shen Fok, Jie
Grumach, Anete S.
Greve, Jens
Guilarte, Mar
Gonçalo, Margarida
Grivcheva Panovska, Vesna
Hide, Michihiro
Hakl, Roman
Jindal, Ankur
Katelaris, Constance H.
Kamaleswaran, Shailajah
Kinaciyan, Tamar
Latysheva, Elena
Larco Sousa, José Ignacio
Lleonart Bellfill, Ramon
Mobayed, Hassan
Metz, Martin
Nasr, Iman
Mitrevska, Natasa T.
Nicola, Stefania
Salvador Parisi, Claudio Alberto
Porebski, Grzegorz
Peter, Jonny
Ferriani, Mariana Paes Leme
Rosario, Nelson
Şekerel, Bülent Enis
Sarquis Serpa, Faradiba
Stobiecki, Marcin
Trainotti, Susanne
Valerieva, Anna
Wongsa, Chamard
Wong, Jane C. Y.
Yucel, Esra
Li, Yinglei
Nenci, Chiara
Maurer, Marcus
Magerl, Markus
Li, Philip H.
author2_role author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
dc.subject.none.fl_str_mv Edema angioneuròtic
Anafilaxi
Angioneurotic edema
Anaphylaxis
topic Edema angioneuròtic
Anafilaxi
Angioneurotic edema
Anaphylaxis
description BackgroundHereditary angioedema (HAE) is a rare and potentially life-threatening genetic disorder characterized by unpredictable attacks of angioedema. MENTALIST (UnMEt Needs in herediTAry angioedema-a gLobal physIcian perSpecTive) is the first international survey uncovering unmet needs and identifying barriers to optimal management in HAE following the latest update of the World Allergy Organization (WAO)/European Academy of Allergy and Clinical Immunology (EAACI) HAE guidelines.MethodsThis web-based survey comprised 24 questions on HAE management and unmet needs. HAE-expert physicians from the Angioedema Centers of Reference and Excellence network ranked unmet needs according to their own perspectives and their patients' perspectives, using a 10-point Likert scale ranging from 0 (not a challenge/unmet need at all) to 10 (huge challenge/unmet need).ResultsOf 64 respondents from 32 countries, most (91%) had > 5 years of experience in managing HAE. Overall, 48% of respondents (n = 31/64) reported that < 50% of their patients had achieved the WAO/EAACI HAE treatment goals of total disease control and normalization of life at the time of the survey. Implementation of consensus recommendations was found to be inconsistent across regions. Gaps in non-HAE-expert physician knowledge, treatment costs, and reimbursement for long-term prophylaxis were the highest-priority challenges according to the respondents. Burden of disease remains a challenge among patients, as reported by their physicians.ConclusionsThe MENTALIST findings highlight a need for removal of barriers to HAE treatment goals and propose a call to action to improve access to treatments, for greater provision of education for physicians and patients, critical collaboration with patient organizations and industry stakeholders and ultimately to optimize HAE care.
publishDate 2025
dc.date.none.fl_str_mv 2025
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://hdl.handle.net/2445/222842
url https://hdl.handle.net/2445/222842
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv Reproducció del document publicat a: https://doi.org/10.1186/s13023-025-03739-8
Orphanet Journal of Rare Diseases, 2025, vol. 20, num. 1
https://doi.org/10.1186/s13023-025-03739-8
dc.rights.none.fl_str_mv cc by (c) Buttgereit, Thomas et al, 2025
http://creativecommons.org/licenses/by/3.0/es/
info:eu-repo/semantics/openAccess
rights_invalid_str_mv cc by (c) Buttgereit, Thomas et al, 2025
http://creativecommons.org/licenses/by/3.0/es/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Springer Science and Business Media LLC
publisher.none.fl_str_mv Springer Science and Business Media LLC
dc.source.none.fl_str_mv Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))
reponame:Dipòsit Digital de la UB
instname:Universidad de Barcelona
instname_str Universidad de Barcelona
reponame_str Dipòsit Digital de la UB
collection Dipòsit Digital de la UB
repository.name.fl_str_mv
repository.mail.fl_str_mv
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