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Iaboni, Aled; Kanani, Amin; Lacuesta, Gina; Song, Christine; Kan, Manstein; Betschel, Stephen D.

BioMed Central

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.

Article

eng

Vancouver : University of British Columbia Library

10.14288/1.0400920

Medicine, Faculty of; Non UBC; Medicine, Department of

10.1186/s13223-021-00579-6

Faculty

http://creativecommons.org/licenses/by/4.0/