Endothelial cell activation during edematous attacks of hereditary angioedema types i and II

Erika Kajdácsi, Péter K. Jani, Dorottya Csuka, Lilian Ágnes Varga, Zoltán Prohászka, Henriette Farkas, László Cervenak

Research output: Contribution to journalArticle

24 Citations (Scopus)


Background Hereditary angioedema (HAE) caused by C1-inhibitor (C1-INH) deficiency (HAE-C1-INH) is a potentially life-threatening rare disease caused by the decreased activity of C1-INH. Lack of C1-INH leads to overproduction of bradykinin, a potent vasoactive peptide. Although angioedema is induced by bradykinin, the function and activation of endothelial cells (ECs), the targets of bradykinin, have not yet been studied during HAE attacks. Objective We studied whether EC function is altered during HAE attacks in comparison with attack-free intervals. Methods Forty-six consecutive samples obtained during attacks from 18 patients with HAE-C1-INH were compared with inter-attack samples of the same patients. The patients' sera were tested for von Willebrand factor (VWF) antigen, VWF collagen-binding activity, soluble E-selectin, and endothelin-1 levels by using ELISA and BRAHMS Kryptor technologies. Results Levels of all 4 EC markers (VWF antigen, VWF collagen-binding activity, soluble E-selectin, and endothelin-1) were significantly increased during HAE attacks. Their increases were even more obvious in the subgroup of patients without any pre-existing risk factors for endothelial dysfunction. Conclusion In this study we demonstrated that ECs are activated during HAE attacks. Our results might suggest the need for revising the knowledge on the pathogenesis of HAE-C1-INH and for reconsidering the role of ECs as a possible novel therapeutic target in patients with this disease.

Original languageEnglish
Pages (from-to)1686-1691
Number of pages6
JournalJournal of Allergy and Clinical Immunology
Issue number6
Publication statusPublished - Jun 2014


  • C1-inhibitor deficiency
  • Hereditary angioedema
  • activation
  • attack
  • clinical study
  • endothelial cells
  • endothelin-1
  • soluble E-selectin
  • von Willebrand factor

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

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