Hereditary angioedema type 1, limitations for prophylactic therapy with Lanadelumab: Regarding a case

Abstract

Background: Hereditary Angioedema is an autosomal dominant disorder caused by a lack or decrease in the function of the C1 inhibitor. It is a rare disease with low prevalence. Treatment focuses on symptom relief and short- and long-term prevention of acute attacks.

Case report: 53-year-old male patient, with recurrent edema in the face, feet, scrotum, associated with abdominal pain since he was 20 years old. Patient has a history of hereditary angioedema in first-degree relatives of consanguinity. Laboratory tests showed low levels of plasma protein (antigenic), functional C1-INH and C4. The patient received several medical treatments. Given the recurrence of symptoms, prophylactic management with Lanadelumab was indicated. However, the patient presented erythema and induration at the application site with subsequent secondary generalized pruritus. For this reason, prophylaxis was suspended.

Conclusion: This case of hereditary angioedema type 1 presents the difficulty in the treatment and the relapse of the patient after trying various therapies, as well as the finding of the adverse effect presented to the prophylactic medication with Lanadelumab, which has been poorly documented in the literature medical worldwide.

Keywords: Hereditary angioedema type 1; Plasmatic protein; Prophylactic management; Adverse event; Lanadelumab.