Abstract

VIEGAS, Leonor Paulos; FERREIRA, Manuel Branco; SANTOS, Amélia Spínola  and  BARBOSA, Manuel Pereira. Hereditary angioedema: Experience with icatibant in severe attacks. Rev Port Imunoalergologia [online]. 2012, vol.20, n.2, pp.129-138. ISSN 0871-9721.

Background:Hereditary angioedema (HAE) is a clinical syndrome characterized by recurrent and transient episodes of submucosal and/or subcutaneous edema. Icatibant, a selective bradykinin B2 receptor antagonist is a new drug available for the treatment of HAE attacks. Aim: Description of our department’s experience with icatibant’s use in severe HAE attacks. Methods: Retrospective study of the patients’ files and telephone interview to the patients admitted between November/2009 and June/2011 to whom icatibant has been administered for acute treatment of severe HAE attacks. Results: Nine patients (5 females; mean age: 30.5 years) were treated with icatibant. Seven had HAE type II. Five patients were admitted due to faringo -laringeal attacks, one of them with associated abdominal complaints, 2 due to exclusive abdominal attacks, and 2 due to exuberant mucocutaneous facial and/or lingual attacks. Laryngoscopy was performed in 7 patients, by ENT specialist, revealing upper airway edema in 3, with regression being documented by laringoscopy in a second ENT evaluation 6 -24 hours later. Every patient reported symptomatic relief in the first 2 hours after subcutaneous administration of 30 mg icatibant. The sole adverse effect mentioned by 88,9% of the patients (8/9), being well tolerated, was a mild pain and/or burn sensation at the injection site. The average time between hospital admission and icatibant’s subcutaneous administration was 2.44 hours, with a median of one hour. Three patients had been previously treated with C1 inhibitor concentrate for past HAE attacks with similar symptoms. These patients reported subjective perception of a shorter time to the beginning of action of icatibant. Conclusions: The authors suggest that icatibant use in acute treatment of severe HAE attacks (faringo -laringeal, abdominal and exuberant mucocutaneous facial/lingual attacks) is effective and safe, associated to minor local, well-tolerated adverse reactions.

Keywords : Bradykinin; hereditary angioedema; icatibant; severe attacks.

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