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Idiopathic Angioedema: Current Challenges

Authors Belbézier A, Bocquet A, Bouillet L

Received 6 August 2019

Accepted for publication 13 March 2020

Published 17 April 2020 Volume 2020:13 Pages 137—144


Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 3

Editor who approved publication: Dr Luis Garcia-Marcos

Aude Belbézier,* Alexis Bocquet,* Laurence Bouillet

National Reference Center for Angioedema (CREAK), Department of Internal Medicine/Clinical Immunology, Grenoble Alpes University Hospital, Grenoble, France

*These authors contributed equally to this work

Correspondence: Laurence Bouillet
Centre de référence des angioedèmes (CREAK), Servicede médecine interne/immunologie clinique, Centre Hospitalier Universitaire de Grenoble Alpes, CS10217, Grenoble cedex 09 38043, France
Tel +33 476765513
Fax +33 76765816

Abstract: The etiological diagnosis of isolated recurrent angioedema poses problems because it must often be done urgently. Angioedema secondary to nonspecific mast cell activation (MC-AE) is the most frequent form and is usually mild. Bradykinin mediated angioedema (BK-AE) is rarer but potentially fatal in the absence of the correct treatment. Few biological markers exist. The C1-inhibitor (C1-inh) functional assay can exclude AE due to C1-inh deficiency. Genetic diagnoses of hereditary AE due to abnormal C1-inh AE have progressed with four currently known mutations. However, determining the physiopathological mechanism leading to some isolated AE cases is sometimes very difficult. In such cases, therapeutic tests are then the only solution: antihistamines at high doses and omalizumab for suspected MC-AE, icatibant for suspected AE-BK. Identifying new markers would be a great help.

Keywords: angioedema, mast cell, bradykinin, C1 inhibitor, histamine

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