Abstract
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.
Publisher
Springer Science and Business Media LLC
Subject
Pulmonary and Respiratory Medicine,Immunology,Immunology and Allergy
Reference17 articles.
1. Nussberger J, Cugno M, Amstutz C, Cicardi M, Pellacani A, Agostoni A. Plasma bradykinin in angio-oedema. Lancet. 1998;351:1693–7.
2. Bork K, Hardt J, Witzke G. Fatal laryngeal attacks and mortality in hereditary angioedema due to C1-INH deficiency. J Allergy Clin Immunol. 2012;130(3):692–7.
3. Betschel S, Badiou J, Binkley K, Borici-Mazi R, Hébert J, Kanani A, et al. The international/Canadian hereditary angioedema guideline. Allergy Asthma Clin Immunol. 2019;15(72):1–29.
4. Bork K, Meng G, Staubach P, Hardt J. Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am J Med. 2006;119:267–74.
5. Lee E, Hsieh J, Borici-Mazi R, Caballero T, Kanani A, Lacuesta G, et al. Quality of life in patients with hereditary angioedema in Canada. Ann Allergy Asthma Immunol. 2021;S1081–1206(21):00002–8. https://doi.org/10.1016/j.anai.2021.01.002.
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