Characteristics of AE attacks
Patients had a median of 2 (±2) attacks at referral. The first attack occurred a median of 2 (±20) months before referral. In at least 6/24 (25%) patients, attacks usually happened in the evening, at night or were noticed when waking up. Prodromes and triggering factors were scarcely reported.
Attacks were mostly localized on the lips (15/30; 50%), tongue (14/30; 47%), throat (9/30; 30%), uvula (3/30; 10%), oral cavity (7/30; 23%), and face (3/10; 10%). Limbs, digestive tract and genitalia attacks were not reported. No patient reported skin lesions of urticaria; and only one mentioned mild pruritus during an attack. ENT symptoms such as dyspnea (4/30; 13%), dysphonia (7/30; 23%) and dysphagia (5/30; 17%) were frequently noted.
Nine patients (30%) reported occurrence of AE attacks that resolved without treatment; and 8 (27%) noted spontaneous resolutions within 24h of AE onset. Classical treatments of histaminergic AE attacks were nearly never reported as effective: antihistamines (1/19; 5%), corticosteroids (1/18; 6%), adrenalin (0/3; 0%). When used, patients seemed to respond to treatments of bradykinin-mediated attacks more consistently: tranexamic acid (7/10; 70%), icatibant (15/19; 79%) and C1-INH concentrate (3/4; 75%). No patient required airway control.