Hereditary angioedema attack: what happens to vasoactive mediators?

Anne Lise Ferrara, Maria Bova, Angelica Petraroli, Nóra Veszeli, Maria Rosaria Galdiero, Mariantonia Braile, Giancarlo Marone, Leonardo Cristinziano, Simone Marcella, Luca Modestino, Henriette Farkas, Stefania Loffredo

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Abstract

Hereditary angioedema is a disabling, life-threatening condition caused by deficiency (type I) or dysfunction (type II) of the C1 inhibitor protein (C1-INH-HAE) leading to bradykinin accumulation and recurrent episodes of edema attack. Vascular leakage is a complex process sustained by the coordinated production of several permeabilizing factors including vascular endothelial growth factors (VEGFs), angiopoietins (ANGPTs) and phospholipase A2 enzymes (PLA2). We previously reported that patients with C1-INH-HAE in remission have increased plasma levels of VEGFs, ANGPTs and secreted PLA2. In this study, we sought to analyze plasma levels of these mediators in 15 patients with C1-INH-HAE during the acute attack compared to remission. Plasma concentrations of VEGF-A, VEGF-C and VEGF-D were not altered during attack compared to remission. Moreover, VEGF-D concentrations were not altered also in remission phase compared to controls. Concentrations of ANGPT1, a vascular stabilizer, were increased during attacks compared to symptoms-free periods, whereas ANGPT2 levels were not altered. The ANGPT2/ANGPT1 ratio was decreased during angioedema attacks. Platelet activating factor acetylhydrolase activity was increased in patients with C1-INH-HAE in remission compared to controls and was decreased during angioedema attacks. Our results emphasize the complexity by which several vasoactive mediators are involved not only in the pathophysiology of C1-INH-HAE, but also during angioedema attacks and its resolution.

Original languageEnglish
Article number106079
JournalInternational Immunopharmacology
Volume78
DOIs
Publication statusPublished - jan. 2020

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ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology
  • Pharmacology

Cite this

Ferrara, A. L., Bova, M., Petraroli, A., Veszeli, N., Galdiero, M. R., Braile, M., Marone, G., Cristinziano, L., Marcella, S., Modestino, L., Farkas, H., & Loffredo, S. (2020). Hereditary angioedema attack: what happens to vasoactive mediators? International Immunopharmacology, 78, [106079]. https://doi.org/10.1016/j.intimp.2019.106079