Hereditary Angioedema: Does It Affect Endothelial Function?

Hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) does not appear to affect endothelial function, according to the results of a recent study.

Because edema formation in C1-INH-HAE is the consequence of interaction nof bradykinin and vasoactive peptides with endothelium, and danazol, used to treat these patients, can affect function of the endothelium, researchers sought to examine rates of endothelial dysfunction in patients with C1-INH-HAE.

They used the flow-mediated dilation method measured in the region of the brachial artery in 33 C1-INH-HAE patients and 30 healthy controls.

Overall, they found no difference in endothelial function between C1-INH-HAE patients and controls. However, elevated cardiovascular risk was observed in danazol-treated C1-INH-HAE patients, but reactive hyperemia values did not differ between danazol-treated and nontreated patients.

“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.”

—Michael Potts

Reference:

Nebenfuhrer Z, Szabo E, Kajdacsi E, et al. Flow-mediated vasodilation assay indicates no endothelial dysfunction in hereditary angioedema patients with C1-inhibitor deficiency [published online October 10, 2018]. Ann Alergy Asthma Immunol. doi: 10.1016/j.anai.2018.10.004.