Efficacy of Different Medical Therapies for the Treatment of Acute Laryngeal Attacks of Hereditary Angioedema due to C1-esterase Inhibitor Deficiency

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• 作者：Konrad Bork ; MD^&lowast ; ; ; Jonathan A. Bernstein ; MD^&dagger ; ; Thomas Machnig ; MD^&Dagger ; ; Timothy J. Craig ; MD^§ ;
• 关键词：C1-INH ; efficacy ; HAE ; laryngeal ; re-dosing
• 刊名：Journal of Emergency Medicine
• 出版年：2016
• 出版时间：April 2016
• 年：2016
• 卷：50
• 期：4
• 页码：567-580.e1
• 全文大小：341 K

文摘

Hereditary angioedema (HAE) is a rare disease characterized by C1-esterase inhibitor (C1-INH) deficiency, resulting in periodic attacks of acute edema, which can be life-threatening if they occur in the upper airway. No head-to-head comparisons of different treatment options for acute HAE attacks are available. Because immediate symptom relief is critical for potentially life-threatening laryngeal attacks, it is important to determine the treatment option that provides optimal treatment response.

Objective

Review and compare data from clinical studies that evaluated the efficacy and safety of treatments for laryngeal HAE attacks.

Methods

We conducted an indirect comparison of clinical outcomes from prospective studies for treatment of 881 acute laryngeal attacks with plasma-derived C1-INH concentrate (pdC1-INH) at fixed doses (500 or 1000 U) or a body weight-adjusted dose (20 U/kg), recombinant C1-INH concentrate at a fixed dose (2100 U), or a body weight-adjusted dose (50 U/kg), icatibant (30 mg), or ecallantide (30 mg). Comparisons included time to onset of symptom relief and need for re-dosing or emergency procedures.

Results

The median time to onset of symptom relief ranged between 15 min and approximately 2 h, and was shortest with body weight-adjusted doses of pdC1-INH. The proportion of laryngeal attacks with re-dosing ranged between 0% and 72%. No re-dosing was needed after treatment with a single body weight-adjusted dose of pdC1-INH (48 attacks).

Conclusions

Available data suggest that among different HAE treatments, body weight-adjusted pdC1-INH (20 U/kg) provides the most reliable treatment response for treatment of laryngeal HAE attacks.