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Allergy, Asthma & Clinical Immunology
Published in: Allergy, Asthma & Clinical Immunology 1/2022

Open Access 01-12-2022 | Angioedema | Case report

Attenuated androgen discontinuation in patients with hereditary angioedema: a commented case series

Authors: Marcus Maurer, Markus Magerl, Emel Aygören-Pürsün, Konrad Bork, Henriette Farkas, Hilary Longhurst, Sorena Kiani‑Alikhan, Laurence Bouillet, Isabelle Boccon-Gibod, Mauro Cancian, Andrea Zanichelli, David Launay

Published in: Allergy, Asthma & Clinical Immunology | Issue 1/2022

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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 Attenuated androgens were either immediately withdrawn, tapered and/or overlapped with another treatment.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.
Appendix
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Metadata
Title
Attenuated androgen discontinuation in patients with hereditary angioedema: a commented case series
Authors
Marcus Maurer
Markus Magerl
Emel Aygören-Pürsün
Konrad Bork
Henriette Farkas
Hilary Longhurst
Sorena Kiani‑Alikhan
Laurence Bouillet
Isabelle Boccon-Gibod
Mauro Cancian
Andrea Zanichelli
David Launay
Publication date
01-12-2022
Publisher
BioMed Central
Published in
Allergy, Asthma & Clinical Immunology / Issue 1/2022
Electronic ISSN: 1710-1492
DOI
https://doi.org/10.1186/s13223-021-00644-0

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