Endothelial cell activation during edematous attacks of hereditary angioedema types I and II - 29/05/14
Abstract |
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.
Le texte complet de cet article est disponible en PDF.Key words : Hereditary angioedema, C1-inhibitor deficiency, attack, endothelial cells, activation, von Willebrand factor, endothelin-1, soluble E-selectin, clinical study
Abbreviations used : BMI, C1-INH, C4, CRP, EC, ET-1, HAE, HAE–C1-INH, sE-selectin, WPB, VWF, VWF:Ag, VWF:CBA
Plan
| Supported by the National Scientific Research Fund (grant no. OTKA K 100886, to H.F.). L.C. was granted the “Bolyai János” Research Fellowship (BO/00218/10/8). |
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| Disclosure of potential conflict of interest: All authors have received research and travel support from the National Scientific Research Fund. |
Vol 133 - N° 6
P. 1686-1691 - juin 2014 Retour au numéro
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