Flow mediated vasodilation assay indicates no endothelial dysfunction in hereditary angioedema patients with C1-inhibitor deficiency

Publication date: Available online 10 October 2018

Source: Annals of Allergy, Asthma & Immunology

Author(s): Zsuzsa Nebenführer, Erika Szabó, Erika Kajdácsi, Kinga Viktória Kőhalmi, István Karádi, András Zsáry, Henriette Farkas, László Cervenak

Abstract

Background

Hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) is a rare, potentially life-threatening disorder characterized by recurrent edematous attacks. The edema formation is the consequence of interaction of bradykinin and various vasoactive peptides with endothelium. Besides these agents, danazol, a modified testosterone derivative used in these patients to prevent edematous attacks, can also affect the function of the endothelium, since it shifts the blood lipid profile to a pro-atherogenic phenotype.

Objective

To assess the endothelial function in C1-INH-HAE patients and in healthy matched controls.

Methods

To evaluate the endothelial function, we used the flow mediated dilation method measured in the region of the brachial artery in 33 C1- INH-HAE patients and in 30 healthy matched controls. Laboratory measurements of standard biochemical parameters were performed on computerized laboratory analyzers.

Results

There was no difference in endothelial function (reactive hyperaemia, RH) between patients (median 9.0, 25-75% percentile 6.3-12.9) and controls (median 7.37, 25-75% percentile 4.52-9.93). Although we found elevated cardiovascular risk (high BMI and LDL/HDL ratio) in danazol treated C1-INH-HAE patients, RH values did not differ between danazol treated and non-treated patients. Furthermore, risk factors correlated with the endothelial function only in healthy controls and patients not treated with danazol.

Conclusion

In summary, our results did not indicate any signs of endothelial dysfunction in C1-INH-HAE patients. Moreover, the normal endothelial function in danazol treated patients with pro-atherogenic lipid profile suggests that elevated bradykinin level or other factor(s) involved in the pathogenesis of edematous attacks may have a protective role against endothelial dysfunction and atherosclerosis.

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