ArticleThe World Allergy Organization journal2025
C1 esterase inhibitor (C1-INH) response as a supportive diagnostic criterion for patients with suspected hereditary angioedema with normal C1-INH.
Article in The World Allergy Organization journal, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Diagnosis of hereditary angioedema (HAE) with normal C1INH level (HAE-nl-C1INH) is based on several criteria, including either an associated genetic variant identified or family history of recurrent angioedema plus lack of high-dose antihistamine response. A rapid, durable response to bradykinin-targeted medication is considered supportive, and this study aimed to evaluate the use of recombinant human C1 esterase inhibitor (rhC1-INH) in this regard. A retrospective medical records review was conducted for angioedema/HAE codes. Thirty-one patients with HAE-nl-C1INH were identified (female, 87.1%; mean age, 46.2 years; range, 16-74 years). All patients had experienced recurrent angioedema, with documentation of antihistamine and/or mast cell-targeted therapy ineffectiveness and normal/near-normal laboratory data. Genetic testing (
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