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Review
. 2024 Dec 9;20(Suppl 3):65.
doi: 10.1186/s13223-024-00934-3.

Angioedema

Affiliations
Review

Angioedema

Gina Lacuesta et al. Allergy Asthma Clin Immunol. .

Abstract

Angioedema can occur in the absence of urticaria and can be broadly divided into three main categories: mast cell-mediated (e.g., histamine), non-mast-cell-mediated (bradykinin-induced) and idiopathic angioedema. Non-mast-cell-mediated angioedema is largely driven by bradykinin. Bradykinin-induced angioedema can be hereditary, acquired or drug-induced, such as with angiotensin-converting enzyme (ACE) inhibitors. Although bradykinin-mediated angioedema can be self-limited, it can cause significant morbidity and laryngeal involvement may lead to fatal asphyxiation. The mainstays of management for angioedema are: (1) to avoid specific triggers (if possible and where known) and (2) treatment with medication (if indicated). For hereditary angioedema (HAE), there are specifically licensed treatments that can be used for the management of attacks, or for prophylaxis in order to prevent attacks. In this article, the authors will review the causes, diagnosis and management of angioedema.

Keywords: Acquired angioedema; Angioedema; Hereditary angioedema.

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Conflict of interest statement

Declarations. Ethics approval and consent to participate: Not applicable. Consent for publication: Not applicable. Competing interests: Dr. Gina Lacuesta has received consulting fees, honoraria and research funding from Takeda, AstraZeneca and Novartis; consulting fees and honoraria from CSL Behring, GSK, Sanofi, Pfizer and Amgen; and consulting fees from BioCryst. Dr. Ellie Tsai has received honoraria and consulting fees from ALK, Astra Zeneca, ICI Medical Communications, Novartis and Sanofi; honoraria from Covis Pharma, CPD Network, GSK, Medexus, Miravo Healthcare and Valeo Pharma; and consulting fees from Bausch Health. Dr. Stephen D. Betschel has received consulting fees, honoraria, and research funding from Astria, Canadian Blood Services, CSL Behring, Green Cross, Grifols, Ionis Pharmaceuticals, Kalvista Novartis, Octapharma, Pharvaris, Sanofi, Takeda, and WSIB. Dr. Harold Kim has received consulting fees from and/or has participated in speakers’ bureau and/or advisory boards for ALK, AstraZeneca, Bausch Health, CSL Behring, GSK, Miravo, Novartis, Pediapharm, Pfizer, Sanofi, Shire, Takeda and Valeo.

Figures

Fig. 1
Fig. 1
Hereditary angioedema (HAE)
Fig. 2
Fig. 2
Mast cell-mediated angioedema
Fig. 3
Fig. 3
Classification of angioedema [1]. *Other drugs like gliptins, neprilysin inhibitors or tissue plasminogen activators are thought to potentially induce bradykinin-mediated AE. AE: angioedema; HAE-1: hereditary angioedema due to C1-inhibitor deficiency; HAE-2: hereditary angioedema due to C1-inhibitor dysfunction; AAE-C1-INH: acquired angioedema due to C1-inhibitor deficiency; HAE-nC1-INH: hereditary angioedema with normal C1-Inhibitor levels, ACEI-AE angiotensin-converting enzyme inhibitor-induced angioedema; IgE, immunoglobulin E Adapted from: Maurer 2022 [1].

References

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    1. Maurer M, Weller K, Bindslev-Jensen C, Giménez-Arnau A, Bousquet PJ, Bousquet J, et al. Unmet clinical needs in chronic spontaneous urticarial. A GA2LEN task force report. Allergy. 2011;66(3):317–30. - PubMed
    1. Zuberbier T, Abdul Latiff AH, Abuzakouk M, Aquilina S, Asero R, Baker D, et al. The international EAACI/GA2LEN/EuroGuiDerm/APAAACI guideline for the definition, classification, diagnosis, and management of urticaria. Allergy. 2022;77(3):734–66. - PubMed
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    1. Jacobs J, Neeno T. The importance of recognizing and managing a rare form of angioedema: hereditary angioedema due to C1-inhibitor deficiency. Postgrad Med. 2021;133(6):639–50. - PubMed

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