dc.contributor.author
Maurer, Marcus
dc.contributor.author
Magerl, Markus
dc.contributor.author
Aygören-Pürsün, Emel
dc.contributor.author
Bork, Konrad
dc.contributor.author
Farkas, Henriette
dc.contributor.author
Longhurst, Hilary
dc.contributor.author
Kiani‑Alikhan, Sorena
dc.contributor.author
Bouillet, Laurence
dc.contributor.author
Boccon-Gibod, Isabelle
dc.contributor.author
Cancian, Mauro
dc.contributor.author
Zanichelli, Andrea
dc.contributor.author
Launay, David
dc.date.accessioned
2023-11-03T16:37:26Z
dc.date.available
2023-11-03T16:37:26Z
dc.identifier.uri
https://refubium.fu-berlin.de/handle/fub188/41419
dc.identifier.uri
http://dx.doi.org/10.17169/refubium-41141
dc.description.abstract
Background: Hereditary angioedema (HAE) is characterized by potentially severe and life-threatening attacks of localized swelling. Prophylactic therapies are available, including attenuated androgens. Efficacy of attenuated androgens has not been assessed in large, randomized, placebo-controlled trials and can be associated with frequent, and sometimes severe, side effects. As better tolerated targeted therapies become available, attenuated androgen withdrawal is increasingly considered by physicians and their patients with HAE. Attenuated androgens withdrawal has not been systematically studied in HAE, although examination of other disorders indicates that attenuated androgen withdrawal may result in mood disturbances and flu-like symptoms. Standardized protocols for attenuated androgen discontinuation that continue to provide control of attacks while limiting potential attenuated androgen withdrawal symptoms are not established as the outcomes of different withdrawal strategies have not been compared. We aim to describe the challenges of attenuated androgen discontinuation in patients with HAE and how these may continue into the post-androgen period.
Case presentation: We present a retrospective case series of 10 patients with confirmed type I HAE who have discontinued prophylactic treatment with attenuated androgens. The most common reason for attenuated androgen discontinuation was side effects. Attenuated androgens were either immediately withdrawn, tapered and/or overlapped with another treatment. The major challenge of discontinuation was the management of an increased frequency and severity of HAE attacks in some patients.
Conclusions: Healthcare teams need to undertake careful planning and monitoring after attenuated androgens discontinuation, and modify treatment strategies if HAE control is destabilized with an increased number of attacks. Discontinuation of attenuated androgens is definitively an option in an evolving HAE treatment landscape, and outcomes can be favourable with additional patient support and education.
en
dc.rights.uri
https://creativecommons.org/licenses/by/4.0/
dc.subject
Attenuated androgens
en
dc.subject.ddc
600 Technik, Medizin, angewandte Wissenschaften::610 Medizin und Gesundheit::610 Medizin und Gesundheit
dc.title
Attenuated androgen discontinuation in patients with hereditary angioedema: a commented case series
dc.type
Wissenschaftlicher Artikel
dcterms.bibliographicCitation.articlenumber
4
dcterms.bibliographicCitation.doi
10.1186/s13223-021-00644-0
dcterms.bibliographicCitation.journaltitle
Allergy, Asthma and Clinical Immunology
dcterms.bibliographicCitation.number
1
dcterms.bibliographicCitation.originalpublishername
Springer Nature
dcterms.bibliographicCitation.volume
18
refubium.affiliation
Charité - Universitätsmedizin Berlin
refubium.funding
Springer Nature DEAL
refubium.resourceType.isindependentpub
no
dcterms.accessRights.openaire
open access
dcterms.bibliographicCitation.pmid
35027083
dcterms.isPartOf.eissn
1710-1492