• Rev Med Interne · Jul 2023

    Review

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

    • D Launay, L Bouillet, I Boccon-Gibod, B Trumbic, D Gobert, Centres coordonnateur et constitutifs CREAK, and O Fain.
    • University Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, 59000 Lille, France; Inserm, 59000 Lille, France; CHU de Lille, service de médecine interne et immunologie clinique, centre de référence angioedèmes à kinine (CREAK), 59000 Lille, France. Electronic address: david.launay@univ-lille.fr.
    • Rev Med Interne. 2023 Jul 1; 44 (7): 344353344-353.

    AbstractHereditary 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.Copyright © 2023 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

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