What is the treatment for angioedema (Angioneurotic Edema)?

From the Guidelines

For the treatment of angioedema, especially in cases of hereditary angioedema, C1 esterase inhibitor concentrates, icatibant, or ecallantide are recommended as first-line treatments, as they have been shown to be effective in reducing the duration and severity of attacks 1.

Treatment Approach

The approach to treating angioedema depends on its severity and cause.

• For mild cases, antihistamines like diphenhydramine (25-50mg every 4-6 hours) or cetirizine (10mg daily) can reduce swelling and itching.
• For moderate to severe cases, oral corticosteroids such as prednisone (40-60mg daily for 3-5 days) may be necessary.
• In emergency situations with airway involvement, immediate medical attention is required, and treatments such as C1-INH, icatibant, or ecallantide should be administered as soon as possible to minimize the duration and severity of the attack 1.

Hereditary Angioedema

For hereditary angioedema, specific medications like C1 esterase inhibitor concentrates, icatibant, or ecallantide may be prescribed by specialists.

• Identifying and avoiding triggers is essential for prevention.
• If angioedema is medication-induced (particularly from ACE inhibitors), discontinuing the offending drug and switching to an alternative is necessary.

Mechanism and Treatment Goal

Angioedema occurs due to the release of inflammatory mediators causing fluid leakage from blood vessels into surrounding tissues.

• Treatment aims to block this inflammatory cascade and reduce swelling until the body can reabsorb the excess fluid.
• The most recent and highest quality study 1 supports the use of icatibant and ecallantide for the treatment of hereditary angioedema, highlighting their efficacy in reducing symptom severity and improving treatment outcomes.

From the FDA Drug Label

1 INDICATIONS AND USAGE KALBITOR® (ecallantide) is indicated for treatment of acute attacks of hereditary angioedema (HAE) in patients 12 years of age and older.

The safety and efficacy of KALBITOR to treat acute attacks of hereditary angioedema in adolescents and adults were evaluated in 2 randomized, double-blind, placebo-controlled trials (EDEMA4 and EDEMA3) in 168 patients with HAE

Treatment of Angioedema:

• The drug KALBITOR (ecallantide) is indicated for the treatment of acute attacks of hereditary angioedema (HAE) in patients 12 years of age and older.
• The recommended dose is 30 mg subcutaneous.
• KALBITOR has been shown to be effective in reducing the severity of symptoms of HAE, with a greater decrease from baseline in the Mean Symptom Complex Severity (MSCS) score and a greater Treatment Outcome Score (TOS) compared to placebo 2, 2.

From the Research

Treatment Options for Angioedema

• The treatment of angioedema depends on the subtype, which can be either histaminergic or non-histaminergic 3, 4, 5.
• For allergic histaminergic angioedema, standard therapies such as epinephrine, glucocorticoids, and antihistamines are effective 3.
• Non-histaminergic angioedema, on the other hand, is often resistant to these drugs and may require alternative treatments 3, 5.
• Therapeutic options for angioedema due to C1-inhibitor deficiencies include C1-inhibitor concentrates, icatibant, and ecallantide 3, 6, 7.
• If these drugs are not available, fresh frozen plasma can be considered as an alternative treatment option 3, 6.

Specific Treatments for ACE Inhibitor-Induced Angioedema

• ACE inhibitor-induced angioedema is a type of non-histaminergic angioedema that does not respond to standard therapies such as epinephrine, glucocorticoids, and antihistamines 5, 6.
• Treatment options for ACE inhibitor-induced angioedema include C1-esterase inhibitor, icatibant, ecallantide, and fresh frozen plasma 6.
• Discontinuation of the causative ACE inhibitor is also recommended as part of the treatment plan 3, 6.

Treatment of Hereditary Angioedema

• Hereditary angioedema due to C1-esterase inhibitor deficiency can be treated with various drugs, including purified plasma-derived human C1 esterase inhibitor concentrate, icatibant acetate, ecallantide, and recombinant human C1 esterase inhibitor 7.
• These drugs can be used for on-demand treatment of acute angioedema attacks, short-term prophylaxis, and long-term prophylaxis 7.
• Other treatment options for hereditary angioedema include tranexamic acid, danazol, and lanadelumab, which can be used for long-term prophylaxis 7.