[Hereditary angioedema and its new treatments: An update].

Abstract

Hereditary angioedema, with or without deficient C1 inhibitor level or function, is a rare disease characterized by recurrent attacks of noninflammatory subcutaneous and/or submucosal edema. It may be life-threatening and substantially affects quality of life. Attacks may be spontaneous or induced, in a setting of emotional stress, by infections or physical trauma, in particular. As the key mediator is bradykinin, this angioedema does not respond to the usual treatments of mast cell-mediated angioedema (antihistamines, corticosteroids, adrenaline), which is much more frequent. Therapeutic management of hereditary angioedema first consists in treating severe attacks with a selective B2 bradykinin receptor antagonist or a C1 inhibitor concentrate. The latter or an attenuated androgen (danazol) can be used for short-term prophylaxis. Therapeutic solutions conventionally proposed for long-term prophylaxis (danazol, antifibrinolytics [tranexamic acid], C1 inhibitor concentrate) vary in efficacy and/or pose problems of safety or ease of use. Kallikrein inhibitors (subcutaneous lanadelumab, oral berotralstat) recently made available as disease-modifying treatment constitute an important advance in long-term prophylaxis of hereditary angioedema attacks. The advent of these new drugs is accompanied by a new ambition for patients: optimize control of the disease and thereby minimize its impact on quality of life.

• Authors: D Launay, L Bouillet, I Boccon-Gibod, B Trumbic, D Gobert, Centres coordonnateur et constitutifs CREAK, O Fain
• Journal: La Revue de medecine interne (Rev Med Interne) Vol. 44 Issue 7 Pg. 344-353 (Jul 2023) ISSN: 1768-3122 [Electronic] France
• Vernacular Title: Mise au point sur les angiœdèmes héréditaires et leurs nouvelles thérapeutiques.
• PMID: 36872215 (Publication Type: English Abstract, Journal Article, Review)
• Copyright: Copyright © 2023 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

Chemical References

• Danazol
• Bradykinin

Topics

• Humans
• Angioedemas, Hereditary (drug therapy)
• Danazol (therapeutic use)
• Quality of Life
• Angioedema (drug therapy)
• Bradykinin (therapeutic use)