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Expert Rev Clin Immunol. 2019 Dec;15(12):1239-1248. doi: 10.1080/1744666X.2020.1693261. Epub 2019 Nov 19.

Lanadelumab for the prevention of attacks in hereditary angioedema.

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Department of Allergology, Medical University of Sofia, Sofia, Bulgaria.
Department of Medicine, University of Padua, Padova, Italy.
Biomedical and Clinical Sciences, University of Milan, Milan, Italy.
Rehabilitation and Internal Medicine Department, IRCCS Istituti Clinici Scientifici Maugeri, Milan, Italy.


Introduction: Hereditary angioedema (HAE) with C1 esterase inhibitor deficiency (C1-INH-HAE) is a rare disease that manifests with cutaneous and/or submucosal swellings due to uncontrolled activation of the contact/kinin system. Attacks recur with unpredictable frequency and severity, laryngeal edema is potentially lethal, and the disease burden may severely disrupt patients' lives.Areas covered: This review provides an overview of lanadelumab, a human monoclonal antibody targeted against plasma kallikrein that was recently approved for prevention of symptoms in C1-INH-HAE.Expert opinion: The phase III HELP Study demonstrated the efficacy of lanadelumab in reducing HAE attacks. These positive results are being further confirmed in the open-label extension study. This agent addresses some of the limitations of existing prophylactic options as tolerability issues, the need for intravenous administration and frequent dosing. Therefore, lanadelumab can profoundly improve the quality of life of patients with C1-INH-HAE.


C1 inhibitor deficiency; Hereditary angioedema; chronic disease; clinical efficacy; cost-effectiveness; lanadelumab; safety; tolerability

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