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

Research output: Contribution to journalArticle

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

Fingerprint

Hereditary Angioedemas
Angioedema
Vascular Endothelial Growth Factors
Vascular Endothelial Growth Factor D
Angiopoietins
Blood Vessels
Complement C1 Inhibitor Protein
Secretory Phospholipase A2
Vascular Endothelial Growth Factor C
Platelet Activating Factor
Phospholipases A2
Bradykinin
Enzymes
Vascular Endothelial Growth Factor A
Edema

Keywords

  • Angiopoietin (ANGPT)
  • Biomarkers
  • Bradykinin
  • Platelet activating factor acetylhydrolase
  • Vascular endothelial growth factor (VEGF)

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., ... Loffredo, S. (2020). Hereditary angioedema attack: what happens to vasoactive mediators? International Immunopharmacology, 78, [106079]. https://doi.org/10.1016/j.intimp.2019.106079

Hereditary angioedema attack : what happens to vasoactive mediators? / Ferrara, Anne Lise; Bova, Maria; Petraroli, Angelica; Veszeli, Nóra; Galdiero, Maria Rosaria; Braile, Mariantonia; Marone, Giancarlo; Cristinziano, Leonardo; Marcella, Simone; Modestino, Luca; Farkas, Henriette; Loffredo, Stefania.

In: International Immunopharmacology, Vol. 78, 106079, 01.2020.

Research output: Contribution to journalArticle

Ferrara, AL, Bova, M, Petraroli, A, Veszeli, N, Galdiero, MR, 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, vol. 78, 106079. https://doi.org/10.1016/j.intimp.2019.106079
Ferrara AL, Bova M, Petraroli A, Veszeli N, Galdiero MR, Braile M et al. Hereditary angioedema attack: what happens to vasoactive mediators? International Immunopharmacology. 2020 Jan;78. 106079. https://doi.org/10.1016/j.intimp.2019.106079
Ferrara, Anne Lise ; Bova, Maria ; Petraroli, Angelica ; Veszeli, Nóra ; Galdiero, Maria Rosaria ; Braile, Mariantonia ; Marone, Giancarlo ; Cristinziano, Leonardo ; Marcella, Simone ; Modestino, Luca ; Farkas, Henriette ; Loffredo, Stefania. / Hereditary angioedema attack : what happens to vasoactive mediators?. In: International Immunopharmacology. 2020 ; Vol. 78.
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