Endothelial cell function in patients with hereditary angioedema: Elevated soluble E-selectin level during inter-attack periods

Judit Czícz, Gyula Schaffer, Dorottya Csuka, Szilvia Walentin, Jan Kunde, Z. Prohászka, H. Farkas, L. Cervenák

Research output: Contribution to journalArticle

15 Citations (Scopus)

Abstract

Background The bradykinin pathway in the pathomechanism of hereditary angioedema due to C1-inhibitor deficiency (henceforward "hereditary angioedema") has been thoroughly studied; however, much less is known about endothelial cell function. Enhanced endothelial cell permeability is obvious during edematous attacks, but not during inter-attack periods. Our knowledge about other endothelial characteristics is even more incomplete. Objective Therefore the aim of this study was to characterize endothelial cell function in hereditary angioedema patients during symptom-free, inter-attack periods. Methods We measured the serum levels of soluble Eselectin, endothelin-1, and von Willebrand factor along with collagen-binding activity in 49 hereditary angioedema patients and in 50 healthy controls. Results Endothelin-1 and von Willebrand factor level, as well as its collagen-binding activity, were similar in hereditary angioedema patients and in controls; however, we found elevated soluble E-selectin levels in the patients. Interestingly, soluble E-selectin concentration did not correlate with any of the inflammatory markers or smoking, and it is not the consequence of the known E-selectin/C1- inhibitor interaction (an analytical phenomenon). In a multiple logistic regression model, the difference in soluble E-selectin between hereditary angioedema patients and controls remained highly significant when adjusted for age, gender, smoking, C-reactive protein, and AB0 blood groups. Conclusion These results demonstrate that in hereditary angioedema, the majority of endothelial functions are normal during inter-attack periods; however, soluble Eselectin levels are elevated. The higher soluble E-selectin plasma concentration is unlikely to result from inflammation; rather, it reflects enhanced shedding mechanisms.

Original languageEnglish
Pages (from-to)61-69
Number of pages9
JournalJournal of Clinical Immunology
Volume32
Issue number1
DOIs
Publication statusPublished - Feb 2012

Fingerprint

Hereditary Angioedemas
E-Selectin
Endothelial Cells
von Willebrand Factor
Endothelin-1
Collagen
Logistic Models
Smoking
Bradykinin
Blood Group Antigens
C-Reactive Protein
Permeability
Inflammation

Keywords

  • C1-inhibitor
  • E-selectin
  • Endothelial function
  • Hereditary angioedema

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Cite this

Endothelial cell function in patients with hereditary angioedema : Elevated soluble E-selectin level during inter-attack periods. / Czícz, Judit; Schaffer, Gyula; Csuka, Dorottya; Walentin, Szilvia; Kunde, Jan; Prohászka, Z.; Farkas, H.; Cervenák, L.

In: Journal of Clinical Immunology, Vol. 32, No. 1, 02.2012, p. 61-69.

Research output: Contribution to journalArticle

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