Successful long-term treatment with the bradykinin B2 receptor antagonist icatibant in a patient with hereditary angioedema.

摘要

BACKGROUND: Hereditary angioedema (HAE) is a rare, autosomal dominant disorder caused by a C1-esterase inhibitor (C1-INH) deficiency (type 1) or qualitative defect (type 2). It is characterized by recurrent subcutaneous or submucosal edema attacks in various organs with a frequency from a few attacks to over one hundred attacks per year. METHODS: We report on the treatment of 141 attacks in a patient with hereditary angioedema with the bradykinin B2 receptor antagonist icatibant. RESULTS: During the entire observation period, efficacy of icatibant was maintained and not altered due to repeated administrations. No systemic or cardiovascular side affects were observed. CONCLUSIONS: In the present case, icatibant proved to be an effective and safe drug for the symptomatic treatment of acute HAE attacks. Its efficacy was not impaired by repeated administrations over a three-year period in this patient.