Publications

Background: Hereditary angioedema (HAE) due to C1-inhibitor deficiency (C1-INH-HAE) is a rare, potentially fatal, bradykinin-mediated disease. Icatibant is a bradykinin B2 receptor antagonist originally approved in 2008 in the EuropeanUnion and 2011 in the United States as an acute therapy option for HAE attacks in adults.

Objective: To compare demographics, disease characteristics and treatment outcomes of icatibant-treated HAEattacks in patients with C1-INH-HAE enrolled in the Icatibant Outcome Survey across six European countries: Austria,France, Germany, Italy, Spain and the UK.

Methods: The Icatibant Outcome Survey [IOS; Shire, Zug, Switzerland (NCT01034969)] is an international observational study monitoring the safety and effectiveness of icatibant. Descriptive, retrospective analyses compared IOS countrydata derived during July 2009–April 2015.

Results: Overall, 481 patients with C1-INH-HAE provided demographic data. A significant difference across countries in age at onset (P=0.003) and baseline attack frequency (P<0.001) was found although no significant differences were found with respect to gender (majority female;P=0.109), age at diagnosis (P=0.182) or delay in diagnosis (P=0.059).Icatibant was used to treat 1893 attacks in 325 patients with majority self-administration in all countries. Overall, significant differences (allP<0.001) were found across countries in time to treatment [median 1.8 h; median range: 0.0(Germany–Austria) to 4.4 (France) h], time to resolution [median 6.5 h; median range: 3 (Germany–Austria) to 12(France) h] and attack duration [median 10.5 h; median range: 3.1 (Germany–Austria) to 18.5 (France) h].

Conclusion: These data form the first European cross-country comparison of disease characteristics and icatibant use in patients with C1-INH-HAE who are enrolled in IOS. International variation in icatibant practice and treatment outcomesacross the six European countries assessed highlight the need to further investigate the range of country-specificparameters driving regional variations in icatibant use.