Methods: We describe two patients with AAE who were treated with icatibant during acute attacks. Patient 1 is an 86 year old male who reported cutaneous and abdominal attacks of angioedema beginning two years earlier with a frequency of 2-3/month. He had low C1-INH (antigenic and functional) C4 levels, normal C1q levels and detectable of anti-C1-INH IgM and anti-C1q IgA. Because of increased frequency, his attacks were treated with icatibant (30 mg s.c.). Patient 2 is a 64 year old female who reported angioedema of the tongue and upper limbs in the last four years. Her C4, C1-INH and C1q were low. Monoclonal gammopathy (6%) and IgA anti C1-INH were found. After the diagnosis she started icatibant for acute attacks.
Results: The first patient used icatibant consecutively for 20 attacks (mainly abdominal), with rapid resolutions of symptoms and no adverse events. Symptoms resolution began 30 minutes after administration of icatibant and resolution was complete in 3-4 hours. The second patient used icatibant for a tongue attack, a mixed cutaneous-tongue attack and a severe facial attack, with first symptom improvement in 25 minutes and complete resolution after 16-18 hours. The only adverse event was erythema at the injection site lasting few minutes.
Conclusions: Icatibant is an effective and well tolerated treatment of acute attacks in patients with AAE. Our data suggest that blocking bradykinin activity could be considered a good therapeutic strategy particularly in patients with anti-C1-INH antibodies.