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Longhurst, HJ; Zanichelli, A; Caballero, T; Bouillet, L; Aberer, W; Maurer, M; Fain, O; Fabien, V; Andresen, I; IOS Study Group.
Comparing acquired angioedema with hereditary angioedema (types I/II): findings from the Icatibant Outcome Survey.
Clin Exp Immunol. 2017; 188(1):148-153
Doi: 10.1111/cei.12910
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PubMed
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- Co-Autor*innen der Med Uni Graz
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Aberer Werner
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- Abstract:
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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.
© 2016 British Society for Immunology.
- Find related publications in this database (using NLM MeSH Indexing)
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Adult -
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Aged -
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Aged, 80 and over -
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Angioedema - diagnosis
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Angioedema - drug therapy
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Angioedema - epidemiology
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Angioedemas, Hereditary - diagnosis
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Angioedemas, Hereditary - drug therapy
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Angioedemas, Hereditary - epidemiology
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Bradykinin - administration & dosage
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Bradykinin - analogs & derivatives
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Bradykinin - therapeutic use
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Disease Progression -
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Female -
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Follow-Up Studies -
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Humans -
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Male -
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Middle Aged -
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Phenotype -
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Retrospective Studies -
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Severity of Illness Index -
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Surveys and Questionnaires -
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Treatment Outcome -
- Find related publications in this database (Keywords)
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acquired angioedema
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hereditary angioedema
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icatibant