Exploring the management of recurrent angioedema caused by different mechanisms

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.

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.

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.