Abstract
C1-inhibitor hereditary angioedema (C1-INH-HAE) is a rare disease characterized by self-limiting edema associated with localized vasodilation due to increased levels of circulating bradykinin. C1-INH-HAE directly influences patients’ everyday lives, as attacks are unpredictable in frequency, severity, and the involved anatomical site. The autonomic nervous system could be involved in remission. The cardiac autonomic profile has not yet been evaluated during the attack or prodromal phases. In this study, a multiday continuous electrocardiogram was obtained in four C1-INH-HAE patients until attack occurrence. Power spectral heart rate variability (HRV) indices were computed over the 4 h preceding the attack and during the first 4 h of the attack in three patients. Increased vagal modulation of the sinus node was detected in the prodromal phase. This finding may reflect localized vasodilation mediated by the release of bradykinin. HRV analysis may furnish early markers of an impending angioedema attack, thereby helping to identify patients at higher risk of attack recurrence. In this perspective, it could assist in the timing, titration, and optimization of prophylactic therapy, and thus improve patients’ quality of life.
Highlights
Angioedema (AE) without wheals is a localized self-limiting edema associated with different mechanisms
On Day 6 of recording, the patient had a mild cutaneous AE attack at approximately 9 a.m., which was relieved by self-administered IV C1-INH within 2 h
In Patient 1, all indices were lower in the ONGOING phase compared to the PRE phase in both Day_Attack and Day_BEFORE_Attack, except for HfnuRR during
Summary
Angioedema (AE) without wheals is a localized self-limiting edema associated with different mechanisms. The best-known form is hereditary angioedema (HAE) due to. C1-inhibitor (C1-INH) deficiency, a rare disease with a prevalence of 1:65,000 in Italy [1]. Symptoms include swelling of the extremities, genitals, bowel mucosa, face, and upper airways, including the larynx. AE attacks are unpredictable and occur episodically upon release of the main mediator of attacks, namely, bradykinin, due to the hyperactivation of the contact system lacking its main control protein, C1-INH [2]. The overall result is an impairment of endothelial function associated with increased vascular permeability [3]. The release of bradykinin occurs locally and unpredictably, at times facilitated by trauma and other triggers such as stress [4,5]
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