Abstract
BackgroundPatients with hereditary angioedema (HAE) due to C1-inhibitor deficiency (C1-INH-HAE) experience recurrent attacks of cutaneous or submucosal edema that may be frequent and severe; prophylactic treatments can be prescribed to prevent attacks. However, despite the use of long-term prophylaxis (LTP), breakthrough attacks are known to occur. We used data from the Icatibant Outcome Survey (IOS) to evaluate the characteristics of breakthrough attacks and the effectiveness of icatibant as a treatment option.MethodsData on LTP use, attacks, and treatments were recorded. Attack characteristics, treatment characteristics, and outcomes (time to treatment, time to resolution, and duration of attack) were compared for attacks that occurred with versus without LTP.ResultsData on 3228 icatibant-treated attacks from 448 patients with C1-INH-HAE were analyzed; 30.1% of attacks occurred while patients were using LTP. Attack rate, attack severity, and the distribution of attack sites were similar across all types of LTP used, and were comparable to the results found in patients who did not receive LTP. Attacks were successfully treated with icatibant; 82.5% of all breakthrough attacks were treated with a single icatibant injection without C1-INH rescue medication. Treatment outcomes were comparable for breakthrough attacks across all LTP types, and for attacks without LTP.ConclusionsPatients who use LTP should be aware that breakthrough attacks can occur, and such attacks can be severe. Thus, patients with C1-INH-HAE using LTP should have emergency treatment readily available. Data from IOS show that icatibant is effective for the treatment of breakthrough attacks. Trial Registration NCT01034969
Highlights
Patients with hereditary angioedema (HAE) due to C1-inhibitor deficiency (C1-INH-HAE) experience recurrent attacks of cutaneous or submucosal edema that may be frequent and severe; prophylactic treatments can be prescribed to prevent attacks
Patient characteristics Data from 3228 icatibant-treated attacks were analyzed from 448 patients with C1-INH-HAE
HAE attacks that occurred with and without long-term prophylaxis (LTP) shared similar characteristics Of the 3228 total icatibant-treated attacks recorded, 973 (30.1%) attacks occurred in 171 patients during LTP (38.2% of the total 448 patients in this analysis), and Patients, N HAE diagnosis, n (%)
Summary
Patients with hereditary angioedema (HAE) due to C1-inhibitor deficiency (C1-INH-HAE) experience recurrent attacks of cutaneous or submucosal edema that may be frequent and severe; prophylactic treatments can be prescribed to prevent attacks. Despite the use of long-term prophylaxis (LTP), breakthrough attacks are known to occur. It is recommended that all patients with C1-INH-HAE have access to therapy that can be used to treat acute angioedema attacks (“on-demand treatment”) [3,4,5,6]. In. Aberer et al Allergy Asthma Clin Immunol (2017) 13:31 addition, patients with C1-INH-HAE may receive longterm prophylaxis (LTP) to reduce the frequency and severity of attacks [5]. Despite the use of LTP, angioedema attacks, some of them severe, have been reported [7,8,9,10,11]. Patients receiving LTP should carry an effective on-demand treatment for acute attacks. There are currently no reports evaluating treatments for breakthrough attacks in detail
Sign-in/Register to view highlights & summary 
Published Version (
Free)
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
