Lanadelumab improves hereditary angioedema attack-free rates in routine clinical practice

Published online December 17, 2024

Hereditary angioedema (HAE) is a rare genetic disease causing painful swelling attacks in parts of the body like the face, hands and feet, and the gut. These attacks often start during childhood or adolescence and recur throughout a patient’s life. Attacks are often unpredictable and disabling, and they can be life threatening if swelling obstructs the airway. Lanadelumab is a treatment given as a regular injection under the skin (subcutaneous) that is used for the routine prevention of recurrent HAE attacks (long-term prophylaxis), aiming to reduce the frequency, severity, and burden of attacks in adults and children aged 2 years and older with HAE. The recommended dosing regimen for patients aged 12 years and older starting lanadelumab treatment is 300 mg every 2 weeks. However, the dosing interval can be extended to once every 4 weeks in patients who are well-controlled on dosing every 2 weeks. Since it is important to understand the effects of new treatments in routine clinical practice, the INTEGRATED study evaluated the effectiveness of long-term lanadelumab treatment in patients with HAE.

Magerl et al. published a manuscript reporting findings from the INTEGRATED study in The Journal of Allergy and Clinical Immunology: In Practice. INTEGRATED is the largest observational study published to date evaluating the effectiveness of lanadelumab in patients with HAE. The study assessed whether starting long-term prophylaxis treatment with lanadelumab in routine clinical practice increased the proportion of patients who were free from attacks.  

The study reviewed the medical records of patients with HAE from 4 European countries (France, Germany, Austria and Greece) who started lanadelumab treatment between August 2018 and May 2021. Data were analyzed from patients in the year before they started lanadelumab and during lanadelumab treatment lasting up to 3.6 years (44 months). The primary outcome of the study was the proportion of patients who had zero attacks (i.e. the attack-free rate) among patients who remained on dosing once every 2 weeks throughout treatment and patients who had individualized treatment with dose interval extensions.

Data from 198 patients with HAE were included in the study. The median time on lanadelumab treatment was 2.4 years (29 months). Most patients (73%) extended lanadelumab dosing intervals during treatment. The average (mean) time to the first extension of the dosing interval was 8.2 months. In the year before treatment, all patients had at least 1 HAE attack, and the average (mean) number of attacks was 35.8 per patient in those 12 months. While patients were receiving lanadelumab treatment, the average (mean) number of attacks was 1.5 per patient per year. Magerl et al. reported a 99% reduction in the average (median) number of attacks that patients had each month. Over 80% of patients in the study were attack-free during the first month of lanadelumab treatment, close to 55% of patients were attack-free for the first year of lanadelumab treatment, and almost 40% remained attack free by the end of the study. Among those who extended lanadelumab dosing intervals, 50% of patients remained attack-free at the end of the study. These findings from the INTEGRATED study suggest that lanadelumab prophylaxis in routine clinical practice can rapidly reduce HAE attacks, with low attack rates over a long period of time, and that a substantial proportion of lanadelumab-treated patients can remain free from HAE attacks.

The Journal of Allergy and Clinical Immunology: In Practice is an official journal of the AAAAI, focusing on practical information for the practicing clinician.

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