title: Comparing acquired angioedema with hereditary angioedema (types I/II): findings from the Icatibant Outcome Survey.
abstract: Icatibant is used to treat acute hereditary angioedema with C1 inhibitor deficiency types I/II (C1-INH-HAE types I/II) and has shown promise in angioedema due to acquired C1 inhibitor deficiency (C1-INH-AAE). Data from the Icatibant Outcome Survey (IOS) were analysed to evaluate the effectiveness of icatibant in the treatment of patients with C1-INH-AAE and compare disease characteristics with those with C1-INH-HAE types I/II. Key medical history (including prior occurrence of attacks) was recorded upon IOS enrolment. Thereafter, data were recorded retrospectively at approximately 6-month intervals during patient follow-up visits. In the icatibant-treated population, 16 patients with C1-INH-AAE had 287 attacks and 415 patients with C1-INH-HAE types I/II had 2245 attacks. Patients with C1-INH-AAE versus C1-INH-HAE types I/II were more often male (69 versus 42%; P = 0·035) and had a significantly later mean (95% confidence interval) age of symptom onset [57·9 (51·33-64·53) versus 14·0 (12·70-15·26) years]. Time from symptom onset to diagnosis was significantly shorter in patients with C1-INH-AAE versus C1-INH-HAE types I/II (mean 12·3 months versus 118·1 months; P = 0·006). Patients with C1-INH-AAE showed a trend for higher occurrence of attacks involving the face (35 versus 21% of attacks; P = 0·064). Overall, angioedema attacks were more severe in patients with C1-INH-HAE types I/II versus C1-INH-AAE (61 versus 40% of attacks were classified as severe to very severe; P < 0·001). Median total attack duration was 5·0 h and 9·0 h for patients with C1-INH-AAE versus C1-INH-HAE types I/II, respectively.
authors.affiliation: Department of Immunology, Barts Health NHS Trust, London, UK.
authors.forename: H J
authors.initials: HJ
authors.lastname: Longhurst
authors.affiliation: Department of Biomedical and Clinical Sciences Luigi Sacco, University of Milan, Luigi Sacco Hospital Milan, Milan, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Zanichelli
authors.affiliation: Department of Allergy, Hospital La Paz Institute for Health Research (IdiPaz), Biomedical Research Network on Rare Diseases (CIBERER, U754), Madrid, Spain.
authors.forename: T
authors.identifier: http://orcid.org/0000-0003-3005-9858
authors.initials: T
authors.lastname: Caballero
authors.affiliation: National Reference Centre for Angioedema, Internal Medicine Department, Grenoble University Hospital, Grenoble, France.
authors.forename: L
authors.initials: L
authors.lastname: Bouillet
authors.affiliation: Department of Dermatology and Venerology, Medical University of Graz, Graz, Austria.
authors.forename: W
authors.initials: W
authors.lastname: Aberer
authors.affiliation: Department of Dermatology and Allergy, Allergie-Centrum-Charité, Charité-Universitätsmedizin Berlin, Berlin, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Maurer
authors.affiliation: Department of Internal Medicine, DHU i2B, Saint Antoine Hospital, University Paris 6, Paris, France.
authors.forename: O
authors.initials: O
authors.lastname: Fain
authors.affiliation: Shire, Zug, Switzerland at the time of data analysis. Now with Vifor Pharma, Glattbrugg, Switzerland.
authors.forename: V
authors.initials: V
authors.lastname: Fabien
authors.affiliation: Shire, Zug, Switzerland.
authors.forename: I
authors.initials: I
authors.lastname: Andresen
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000799:Angioedema / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology; D054179:Angioedemas, Hereditary / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology; D001920:Bradykinin / Q000008:administration & dosage / Q000031:analogs & derivatives* / Q000627:therapeutic use; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D012189:Retrospective Studies; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires; D016896:Treatment Outcome
chemical_list: C065679:icatibant; D001920:Bradykinin
keywords: acquired angioedema; hereditary angioedema; icatibant