Exploring the management of recurrent angioedema caused by different mechanisms.

Purpose of review: We aim to explore the most recent insights into the pathogenesis of recurrent angioedema caused by different mechanisms and then focus on the management and treatment approaches available.

Recent findings: The recently developed DANCE consensus classification identifies five types of angioedema: mast cell-mediated (AE-MC), bradykinin-mediated, because of intrinsic vascular endothelium dysfunction (AE-VE), drug-induced (AE-DI), and due to unknown mechanisms (AE-UNK). These subtypes require different management with treatment choices targeting the main pathogenetic pathways involved in each form. For AE-MC and AE-BK, the therapeutic landscape has been significantly widened in recent years. Conversely, there is a lack of consensus for the hereditary forms because of newly discovered mutations ( factor 12 , plasminogen, kininogen-1 , myoferlin, angiopoietin-1 , heparan sulfate 3-O-sulfotransferase 6 ) and AE-UNK.

Summary: Recurrent angioedema can present with or without wheals. Angioedema without wheals may be driven by bradykinin and/or mast cell mediators. The different forms respond to specific drugs and require a different management. For its potentially life-threatening and disfiguring features, angioedema should be promptly recognized and effectively treated. For this reason, enhancing awareness about various angioedema subtypes and their management provide a useful tool for the clinical practice.