Guidance for diagnosis and treatment of acute angioedema in the emergency department: consensus statement by a panel of Italian experts

Abstract

Angioedema attacks, characterized by the transient swelling of the skin and mucosae, are a frequent cause of visits to the emergency department. Swellings of the oral cavity, tongue, or larynx can result in life-threatening airway obstruction, while abdominal attacks can cause severe pain and often lead to unnecessary surgery. The underlying pathophysiologic process resulting in increased vascular permeability and plasma extravasation is mediated by vasoactive molecules, most commonly histamine and bradykinin. Based on the mediator involved, distinct angioedema forms can be recognized, calling for distinct therapeutic approaches. Prompt recognition is challenging for the emergency physician. The low awareness among physicians of the existence of rare forms of angioedema with different aetiologies and pathogenesis, considerably adds to the problem. Also poorly appreciated by emergency personnel may be the recently introduced bradykinin-targeted treatments. The main objective of this consensus statement is to provide guidance for the management of acute angioedema in the emergency department, from presentation to discharge or hospital admission, with a focus on identifying patients in whom new treatments may prevent invasive intervention.

Presentation, diagnosis and treatment of angioedema without wheals: a retrospective analysis of a cohort of 1058 patients

“…According to recently published criteria [3], diagnosis of HAE was dependent on a history of recurrent angioedema in a first-or second-degree relative, or the presence of an angioedema-associated mutation in SERPING1 or F12. Diagnosis of C1-INH deficiency required evidence of either antigenic or functional plasma levels of at least 50% below the normal value.…”

“…The condition can be acute, mainly due to an allergic or pseudoallergic reaction to foods or drugs, or recurrent. With regard to the latter, it is important to distinguish between angioedema occurring with wheals, which is part of the clinical presentation of urticaria, and angioedema without wheals, which is a separate patho- Original Article [2], whereas angioedema types were classified very recently [3]. Accordingly, recurrent angioedema without wheals can be categorized as hereditary (HAE) and acquired (AAE) forms.…”

Recurrent Angioedema: Occurrence, Features, and Concomitant Diseases in an Italian Single-Center Study

“…Recent evidence suggests novel laboratory methods for the differential diagnosis of AAE and HAE when the determination of C1-INH is not yet available [56] . There is widespread agreement on therapeutic goals (i.e., to reduce mortality and morbidity, reduce the frequency of attacks, improve quality of life, and train patients in selfadministration) in C1-INH-related AE whereas no guidelines are available concerning the treatment of ACEI-RA and I-AAE [22,23,[57][58][59] .…”

Treatment of ACEI-related angioedema with icatibant: a case series

“…Seven patients were on enalapril 20 mg/day, three on ramipril 5 mg/day (one of these patients was also on vidagliptin, an antidiabetic drug that is also rarely associated with an increased risk of angioedema among patients taking an ACEI), two were on lisinopril 20 mg/day, and one on quinapril 20 mg/day [22]. All the patients presented with angioedema symptoms, and were first treated with standard therapy based on corticosteroids (all patients), antihistamine drugs (10 patients), and epinephrine (7 patients) [23]. Due to the lack of response to standard therapy, and the worsening and severity of symptoms, all patients received one injection of subcutaneous icatibant (30 mg per 3 mL in a prefilled syringe).…”