Abstract
BackgroundAttacks of Hereditary Angioedema due to C1-inhibitor deficiency (C1-INH-HAE)are often triggered by stressful events/hormonal changes.ObjectiveOur study evaluates the relationship between autonomic nervous system (ANS) and contact/complement system activation.MethodsTwenty-three HAE patients (6 males, mean age 47.5±11.4 years) during remission and 24 healthy controls (8 males, mean age 45.3±10.6 years) were studied. ECG, beat-by-beat blood pressure, respiratory activity were continuously recorded during rest (10’) and 75-degrees-head-up tilt (10’). C1-INH, C4, cleaved high molecular weight kininogen (cHK) were assessed; in 16 patients and 11 controls plasma catecholamines were also evaluated. Spectral analysis of heart rate variability allowed extraction of low-(LF) and high-(HF) frequency components, markers of sympathetic and vagal modulation respectively.ResultsHAE patients showed higher mean systolic arterial pressure (SAP) than controls during both rest and tilt. Tilt induced a significant increase in SAP and its variability only in controls. Although sympathetic modulation (LFnu) increased significantly with tilt in both groups, LF/HF ratio, index of sympathovagal balance, increased significantly only in controls.At rest HAE patients showed higher noradrenaline values (301.4±132.9 pg/ml vs 210.5±89.6pg/ml, p = 0.05). Moreover, in patients tilt was associated with a significant increase in cHK, marker of contact system activation (49.5 ± 7.5% after T vs 47.1 ± 7.8% at R, p = 0.01).ConclusionsOur data are consistent with altered ANS modulation in HAE patients, i.e. increased sympathetic activation at rest and blunted response to orthostatic challenge. Tilt test-induced increased HK cleavage suggests a link between stress and bradykinin production.
Highlights
Angioedema, is a localized self-limiting edema associated with different mechanisms
Tilt induced a significant increase in systolic arterial pressure (SAP) and its variability only in controls
In patients tilt was associated with a significant increase in cleaved high molecular weight kininogen (cHK), marker of contact system activation (49.5 ± 7.5% after T vs 47.1 ± 7.8% at R, p = 0.01)
Summary
Angioedema, is a localized self-limiting edema associated with different mechanisms It can arise with wheals in a setting of allergic reactions or of chronic spontaneous urticarial as a result of mast cell degranulation. Symptoms occur episodically upon release of bradykinin resulting from hyperactivation of the contact system lacking its main control protein C1-INH [2] They can affect cutaneous districts, as well as the gastrointestinal and upper airway mucosa. Angioedema can be insignificant, but most of the time it causes disability with personal and social consequences such as disfiguration and severe abdominal pain When it localizes to the larynx, it can be lethal and more than 25% of affected subjects suffocate if appropriate treatments are not immediately available [3]. Attacks of Hereditary Angioedema due to C1-inhibitor deficiency (C1-INH-HAE)are often triggered by stressful events/hormonal changes
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