Idiopathic Angioedema: Current Challenges

• Main Authors: Belbézier A, Bocquet A, Bouillet L
• Format: Article
• Language: English
• Published: Dove Medical Press 2020-04-01
• Series: Journal of Asthma and Allergy
• Subjects: angioedema; mast cell; bradykinin; c1inhibitor; histamine
• Online Access: https://www.dovepress.com/idiopathic-angioedema-current-challenges-peer-reviewed-article-JAA

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 workCorrespondence: Laurence BouilletCentre de référence des angioedèmes (CREAK), Servicede médecine interne/immunologie clinique, Centre Hospitalier Universitaire de Grenoble Alpes, CS10217, Grenoble cedex 09 38043, FranceTel [phone]Fax [phone]Email [e-mail]: 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