What is the management of Angioedema (Angioneurotic Edema)?

From the Guidelines

The management of angioedema should prioritize the use of specific treatments such as C1 esterase inhibitor concentrates, icatibant, or ecallantide for hereditary or bradykinin-mediated angioedema, as these have been shown to be safe and efficacious in treating acute attacks 1. The primary goal in managing angioedema is to ensure airway patency and alleviate symptoms.

• For hereditary angioedema (HAE), the use of epinephrine, corticosteroids, and antihistamines is not recommended as they are not efficacious in treating HAE attacks 1.
• Instead, on-demand treatment with C1INH concentrates, a plasma kallikrein inhibitor, or a bradykinin B2 receptor antagonist is preferred for acute HAE attacks 1.
• Symptomatic treatment based on the region of body swelling can also be involved in managing HAE attacks, including pain management and hydration for abdominal attacks, and maintaining airway patency for oropharyngeal and laryngeal attacks 1.
• Long-term management involves identifying and avoiding triggers, carrying emergency medications, and considering prophylactic options like danazol, tranexamic acid, or lanadelumab for hereditary angioedema. Key considerations in managing angioedema include:
• Prompt treatment to prevent life-threatening airway obstruction
• Individualized management based on the patient's situation and the severity of the attack
• Avoidance of unnecessary medications that may worsen symptoms or have adverse effects
• Close monitoring for signs and symptoms of impending airway closure in patients with oropharyngeal or laryngeal attacks.

From the FDA Drug Label

Icatibant injection is a bradykinin B2 receptor antagonist indicated for treatment of acute attacks of hereditary angioedema (HAE) in adults 18 years of age and older. 30 mg injected subcutaneously in the abdominal area. If response is inadequate or symptoms recur, additional injections of 30 mg may be administered at intervals of at least 6 hours. Do not administer more than 3 injections in 24 hours.

The management of Angioedema (specifically hereditary angioedema, HAE) involves the administration of icatibant injection.

• The recommended dose is 30 mg injected subcutaneously in the abdominal area.
• If the response is inadequate or symptoms recur, additional injections of 30 mg may be administered at intervals of at least 6 hours.
• The maximum number of injections allowed in 24 hours is 3.
• Patients may self-administer upon recognition of an HAE attack 2.
• It is also advised that following treatment of laryngeal attacks with icatibant injection, patients should seek immediate medical attention 2.

From the Research

Management of Angioedema

The management of angioedema involves a step-by-step approach to diagnose and treat the condition effectively. The following are the key points to consider:

• Initial evaluation of consciousness and vital signs to manage breathing and support circulation if necessary 3
• Differentiation between histaminergic and non-histaminergic angioedema, with concomitant urticaria and pruritus suggesting a histaminergic origin 3
• Investigation of exposure to allergens and drugs, such as ACE inhibitors and non-steroidal anti-inflammatory drugs, as well as family history of similar symptoms 3

Treatment Options

The treatment options for angioedema depend on the underlying cause and can be categorized into:

• Allergic histaminergic angioedema: sensitive to standard therapies such as epinephrine, glucocorticoids, and antihistamines 3, 4
• Non-histaminergic angioedema: often resistant to standard therapies, with therapeutic options including C1-inhibitor concentrates, icatibant, and ecallantide 3, 4
• ACE inhibitor-induced angioedema: discontinuation of the causative drug is recommended, with variable results from medications such as icatibant, ecallantide, and C1 inhibitor replacement 3, 5

Airway Management

Airway management is crucial in cases of angioedema, particularly when there is involvement of the oral cavity and larynx. The following are key points to consider:

• Evaluation of the airway and preparation for fiberoptic or video laryngoscopy, with cricothyrotomy if necessary 4
• Disposition dependent on patient's airway and respiratory status, as well as the sites involved 4

Pharmacotherapy

Pharmacotherapy for angioedema can be challenging, particularly in cases of ACE inhibitor-induced angioedema. The following are key points to consider:

• Limited evidence supporting the efficacy of pharmacotherapy for ACEI-induced angioedema, with variable results from medications such as icatibant, ecallantide, and C1 inhibitor replacement 5
• Fresh frozen plasma may be considered as an alternative treatment option for progressive and refractory ACEI-induced angioedema 6