Impact of lanadelumab in hereditary angioedema: a case series of 12 patients in Canada

Abstract

Background Hereditary angioedema (HAE) is a rare autosomal dominant disease resulting in recurring episodes of swelling, leading to considerable patient morbidity and mortality. Lanadelumab is a plasma kallikrein inhibitor that is approved as 1st line therapy in Canada for long term prophylaxis of HAE attacks. Objective To describe our clinical findings from a case series of adult patients with HAE type 1/2 who have been initiated on lanadelumab. Methods A chart review of HAE type 1/2 patients at three academic centers in Canada was undertaken with demographic and clinical data extracted. Patients were included if they had been receiving lanadelumab for at least 6 months. Patients with other causes of angioedema were excluded. Results 12 patients meeting enrollment criteria were identified. Compared to pre-lanadelumab, patients had mean reductions of 72% and 62% in attack rate and treated attack rate respectively. 3 patients reported complete remission from attacks after starting lanadelumab. Most patients had significant improvements in HAE impact on social outings. Conclusion Our case series findings support the 2019 International/Canadian HAE guideline that lanadelumab is an effective therapy for long term prophylaxis. In our patient population, initiation of lanadelumab improved disease control, minimized the burden of treatment and improved HAE impact on social outings.

Angioedema and Fatty Acids

“…A recently updated understanding of angioedema helped to develop novel therapeutic options, especially lanadelumab and berotralstat, which target the upstream factors, accelerating bradykinin production. Lanadelumab is a human-specific monoclonal antibody targeting plasma kallikrein that acts as an inhibitor and reduces downstream bradykinin generation; several studies showed that lanadelumab potentiates the therapeutic preventive effect for angioedema development [29][30][31][32]. Berotralstat suppresses the development of angioedema by the inhibitory action of plasma kallikrein, subsequently decreases bradykinin production, and shows strong regulatory action for angioedema; this efficacy was confirmed by several clinical studies [33][34][35][36].…”

Using an extended treatment regimen of lanadelumab in the prophylaxis of hereditary angioedema: a single-centre experience

“…All our patients reported a significant improvement in the number of angioedema attacks per month, which is in line with previous real-life reports. 1 , 8 , 9 Moreover, over half of the patients experienced complete remission from attacks since starting lanadelumab. Patients also reported a significant improvement in their quality of life after starting lanadelumab.…”

“… 8 Moreover, Iaboni et al reported that two-thirds of their patients reported no impact on social outings due to angioedema attacks, with only 1 patient having to miss work, after at least 6 months of lanadelumab. 9 …”

“…7 Real-life reports have shown that it is a safe and effective prophylactic agent, however treatment cost is high. 1 , 8 , 9 Using an extended dosing regimen (every 4 weeks) may prove helpful in difficult-to-treat patients who cannot afford the standard regimen (every 2 weeks). We report a single-center experience to compare the effectiveness and safety profile of an extended dosing regimen, compared to a standard regimen, of lanadelumab in HAE.…”

Successful Use of Lanadelumab in an Older Patient With Type II Hereditary Angioedema

“…5 Also, no patients aged ≥ 65 years were part of the treatment arms that included a lanadelumab dose of 300 mg. 5 In a case series of 12 patients in Canada, treatment with lanadelumab decreased angioedema attacks by mdedge.com/fedprac 72%. 9 However, the series only included 1 patient with type II HAE who was aged 36 years. 9 Therefore, our case demonstrates the efficacy of lanadelumab in a patient aged ≥ 65 years with type II HAE.…”