Veröffentlichungen

Icatibant is used to treat acute hereditary angioedema with C1 inhibitordeficiency types I/II (C1-INH-HAE types I/II) and has shown promise inangioedema due to acquired C1 inhibitor deficiency (C1-INH-AAE). Datafrom the Icatibant Outcome Survey (IOS) were analysed to evaluate theeffectiveness of icatibant in the treatment of patients with C1-INH-AAE andcompare disease characteristics with those with C1-INH-HAE types I/II. Keymedical history (including prior occurrence of attacks) was recorded uponIOS enrolment. Thereafter, data were recorded retrospectively atapproximately 6-month intervals during patient follow-up visits. In theicatibant-treated population, 16 patients with C1-INH-AAE had 287 attacksand 415 patients with C1-INH-HAE types I/II had 2245 attacks. Patientswith C1-INH-AAEversusC1-INH-HAE types I/II were more often male (69versus42%;P50035) and had a significantly later mean (95% confidenceinterval) age of symptom onset [579 (5133–6453)versus140 (1270–1526) years]. Time from symptom onset to diagnosis was significantlyshorter in patients with C1-INH-AAEversusC1-INH-HAE types I/II (mean123 monthsversus1181 months;P50006). Patients with C1-INH-AAEshowed a trend for higher occurrence of attacks involving the face (35versus21% of attacks;P50064). Overall, angioedema attacks were more severe inpatients with C1-INH-HAE types I/IIversusC1-INH-AAE (61versus40% ofattacks were classified as severe to very severe;P<0001). Median totalattack duration was 50hand90 h for patients with C1-INH-AAEversusC1-INH-HAE types I/II, respectively.