D1.384 - Long-term Prophylaxis with Lanadelumab in Hereditary and Acquired Angioedema: A Single-Center Retrospective-Prospective “Real-Life” Analysis

Hereditary angioedema (HAE) is a rare disease characterized by recurrent episodes of mucocutaneous edema, with severity ranging from mild to potentially life-threatening. Onco-hematologic and autoimmune disorders can lead to a similar condition, known as acquired angioedema (AAE).Lanadelumab, a human monoclonal antibody (IgG1-Kappa) that inhibits active plasma kallikrein, is used for long-term prophylaxis (LTP) of this disease.We retrospectively evaluated the long-term efficacy and safety of Lanadelumab in a cohort of patients with HAE and AAE.

Thirteen patients (5M:8F; 11 HAE and 2 AAE) undergoing therapy with Lanadelumab 300 mg/14 days (4 patients since 2021, 4 since 2022, and 5 since 2024) were monitored for attack frequency, disease activity (AECT), and adverse events at 6 and 12 months.

The pre-treatment attack frequency was 36.76 ± 29 per year, with a disease activity AECT score of 6 ± 2/16. After six months, all patients showed a reduction in attack frequency (0.76 ± 1.17), corresponding to a 97.8% decrease, along with a significant improvement in disease activity (AECT 15 ± 1). At 12 months, Lanadelumab’s efficacy was sustained, with an attack frequency of 0.77 ± 1.7 per year and an AECT score of 15 ± 1.Comparing patients who started therapy in 2021 with those who initiated treatment in 2022 and 2024, no statistically significant differences were observed, suggesting that, in our cohort, the results achieved at 12 months were maintained up to 24, 36, and 48 months.Only one patient experienced a mild adverse event (erythema at the injection site).

In our cohort, Lanadelumab proved to be an effective and safe treatment for HAE and AAE, demonstrating a rapid and sustained therapeutic response over time.