Clinical similarities among bradykinin‐mediated and mast cell‐mediated subtypes of non‐hereditary angioedema: a retrospective study

Abstract

BackgroundNon-hereditary angioedema (non-HAE) is characterized by local swelling due to self-limiting, subcutaneous or submucosal extravasation of fluid, and can be divided into three subtypes. These subtypes are believed to have different pathophysiological backgrounds and are referred to in recent guidelines as bradykinin-mediated (e.g. caused by angiotensin-converting-enzyme-inhibitors), mast cell-mediated (e.g. angioedema with wheals) or idiopathic (cause unknown). Bradykinin-mediated subtypes are more closely related to hereditary angioedema than the other forms. Because clinical features of these non-HAE subtypes have not been studied in detail, we have looked at the clinical characteristics of symptoms and potential differences in clinical presentation of bradykinin-mediated and mast cell-mediated angioedema (AE) subtypes.MethodsA questionnaire was sent to patients presenting with AE at our tertiary outpatient clinic to document clinical characteristics, potential triggers and location of AE. The severity of AE attacks was analysed using visual analogue scales (VAS).ResultsThe questionnaire was returned by 106 patients, of which 104 were included in the analysis. AE with wheals, idiopathic AE, and drug-associated AE occurred in 64 (62%), 25 (24%) and 15 patients (14%) respectively. Most patients (62%) reported prodromal symptoms while 63% reported multiple locations for an attack. Face and oropharynx were the main locations of AE attacks of any subtype while swelling was the symptom most frequently reported as severe. Overall severity of the last attack was indicated as severe by 68% of the patients. There were no differences between the subgroups.ConclusionThis similarity in clinical presentation raises the possibility that ACEi-induced, mast cell-mediated and idiopathic AE share common pathways.Electronic supplementary materialThe online version of this article (doi:10.1186/s13601-015-0049-8) contains supplementary material, which is available to authorized users.

How to control recurrent angioedema using monoclonal antibody therapies?

“…Angioedema of the upper throat may cause breathing difficulties, however, suffocation does not occur. Some CSU patients with RA have mild or moderate recurrent abdominal discomfort [11], but abdominal swelling attacks do not occur.…”

Adverse Drug Reactions in the ICU

“…Angioedema, or rapid localized edema of the deep dermis, subcutaneous, or submucosal tissues, can be idiopathic, or it can be mediated by bradykinin or mast cells [38]. Angioedema associated with the use of drugs can manifest after the first dose of a drug, but for some drugs, such as those targeting the renin-angiotensin-aldosterone system, it can occur at any time [39].…”

“…Culprit medications include nonsteroidal anti-inflammatory drugs (NSAIDs) or antibiotics, often acting through the inhibition of cyclooxygenase resulting in alteration in the metabolism of arachidonic acid with increased leukotrienes [40][41][42]. Angioedema without wheals or urticaria could be bradykinin mediated, which implicates angiotensinconverting enzyme inhibitors [38]. Bradykinin Abbreviations: calcium (Ca), chloride (Cl), immunoglobulin (Ig), and potassium (K ) accumulation results in an increased vascular permeability resulting in angioedema [42].…”

Efficacy of Treatment of Non-hereditary Angioedema

“…Angioedema (AE) frequently occurs as part of urticaria, a disease characterized by the development of wheals, AE, or both [ 1 , 2 ]. AE with wheals, also known as chronic spontaneous urticaria (CSU), is presumably mast cell-mediated [ 1 – 3 ]. AE without significant wheals can be the presenting symptom of a variety of diagnoses, such as hereditary AE caused by C1 esterase inhibitor (C1INH) deficiency, resulting in the release of the key mediator bradykinin [ 2 ].…”

“…ACEi-AE is estimated to occur in up to 0.68 % of patients who receive ACE inhibitors [ 5 ]. However, a majority of patients suffer idiopathic acquired AE, which implies AE with normal C1INH with no family history of AE, in which known causes of AE have been excluded [ 2 , 3 ]. It is unclear to what extent idiopathic AE is similar to angioedema with wheals (CSU) [ 3 ], or to presumably bradykinin-mediated subtypes of AE.…”