This study was funded by CSL Behring GmbH Marburg, Germany.
PK and HOK conceived the study and designed the trial. TJC, RLW, RJL, AKB, LS, FP, and WY undertook recruitment of patients and managed data, including quality control. HOK performed the statistical analyses. TJC, RLW, RJL, AKB, LS, FP, WY, HOK, and PK provided analysis and interpretation of data and contributed to preparation of the manuscript. TJC takes responsibility for the manuscript as a whole.
Hereditary angioedema (HAE) is a rare disorder characterized by C1 esterase inhibitor (C1-INH) deficiency, resulting in periodic attacks of acute edema that can be life-threatening if they occur in the laryngeal region. We assessed the efficacy of C1-INH concentrate in the emergency treatment of rarely occurring acute laryngeal HAE attacks in a prospective, open-label clinical study.
Acute laryngeal attacks were each treated with C1-INH concentrate (Berinert) at a single dose of 20 U/kg body weight. Efficacy endpoints included time to onset of symptom relief and time to complete resolution of all symptoms, each based on the patient's assessment.
All 39 laryngeal attacks in 16 patients were treated successfully. The median time to onset of symptom relief was 15 min. The median time to complete resolution of all symptoms was 8.25 h. No treatment-related serious adverse events occurred, and the treatment was well tolerated. The administration of C1-INH concentrate was not associated with any viral infections.
C1-INH concentrate is an effective and safe emergency treatment for providing reliable and rapid relief from the potentially life-threatening symptoms of laryngeal HAE attacks.
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- Prospective Study of Rapid Relief Provided by C1 Esterase Inhibitor in Emergency Treatment of Acute Laryngeal Attacks in Hereditary Angioedema
Timothy J. Craig
Richard L. Wasserman
Robyn J. Levy
Againdra K. Bewtra
William H. Yang
Peter C. Kiessling
- Springer US
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