What is the treatment for Angioedema (Angioneurotic Edema)?

From the Guidelines

Angioedema treatment should prioritize the use of C1-INH, icatibant, or ecallantide for acute attacks, with early treatment being crucial to minimize duration and severity, as recommended by guidelines 1.

Key Considerations

• The primary mediator of swelling in patients with hereditary angioedema (HAE) is bradykinin, and standard angioedema treatment modalities like epinephrine, corticosteroids, or antihistamines are not efficacious and not recommended for HAE treatment 1.
• Fresh frozen plasma might be effective in abrogating HAE attacks but can also acutely exacerbate some attacks, requiring caution and consideration of alternative safer medications 1.
• Management of HAE attacks can involve symptomatic treatment based on the region of body swelling, with a focus on maintaining airway patency in oropharyngeal and laryngeal attacks 1.

Treatment Approach

• For hereditary angioedema, specific medications like C1 esterase inhibitor concentrates (Berinert, Cinryze), icatibant (Firazyr), or ecallantide (Kalbitor) are used, with guidelines emphasizing the need for early treatment and consideration of intubation or tracheotomy in upper airway angioedema 1.
• Long-term prophylaxis for recurrent angioedema may include danazol, tranexamic acid, or regular C1 inhibitor infusions.
• Angiotensin-converting enzyme (ACE) inhibitor-induced angioedema requires discontinuation of the medication and switching to alternative antihypertensives.

Important Notes

• The treatment approach targets the underlying mechanism of swelling, which involves excessive release of inflammatory mediators causing increased vascular permeability and fluid accumulation in tissues.
• Patients with HAE should carry an acute attack treatment on their person and be trained on self-administration, with the exception of ecallantide 1.

From the FDA Drug Label

BERINERT is a plasma-derived C1 Esterase Inhibitor (Human) indicated for the treatment of acute abdominal, facial, or laryngeal hereditary angioedema (HAE) attacks in adult and pediatric patients. The recommended treatment for angioedema is C1 esterase inhibitor (IV), specifically BERINERT, which is indicated for the treatment of acute abdominal, facial, or laryngeal hereditary angioedema (HAE) attacks in adult and pediatric patients 2.

• The safety and efficacy of BERINERT in the treatment of acute abdominal or facial HAE attacks in subjects with hereditary angioedema were demonstrated in a placebo-controlled, double-blind, prospective, multinational, randomized, parallel-group, dose-finding, three-arm, clinical study 2.
• BERINERT was shown to shorten the time to onset of relief of symptoms of an abdominal or facial HAE attack compared to placebo 2.
• The clinical study assessed the efficacy and safety of BERINERT in 124 adult and pediatric subjects with C1 esterase inhibitor deficiency who were experiencing an acute moderate to severe attack of abdominal or facial HAE 2.

From the Research

Angioedema Treatment Overview

• Angioedema is a self-limiting edema of the subcutaneous or submucosal tissues due to localized increase of microvascular permeability whose mediator may be histamine or bradykinin 3.
• The treatment of angioedema depends on the subtype, which can be either histaminergic or non-histaminergic 3, 4.

Histaminergic Angioedema Treatment

• Allergic histaminergic angioedema is sensitive to standard therapies such as epinephrine, glucocorticoids, and antihistamines 3.
• Concomitant urticaria and pruritus suggest a histaminergic origin, and exposure to allergens and drugs should be investigated 3.

Non-Histaminergic Angioedema Treatment

• Non-histaminergic angioedema is often resistant to standard therapies such as epinephrine, glucocorticoids, and antihistamines 3.
• Therapeutic options for angioedema due to C1-inhibitor deficiencies are C1-inhibitor concentrates, icatibant, and ecallantide 3.
• Fresh frozen plasma can be considered if these drugs are not available 3, 5, 6.

ACE Inhibitor-Induced Angioedema Treatment

• ACE inhibitor-induced angioedema is a well-recognized adverse effect of angiotensin-converting enzyme inhibitors 5, 7, 6.
• Conventional treatment may be of limited benefit due to the mechanism of the reaction, and C1 esterase inhibitors have been utilized in treating ACE inhibitor-induced angioedema 7, 6.
• Discontinuation of the causative drug is recommended 3.