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  • Title: Contact System Activation and Bradykinin Generation in Angioedema: Laboratory Assessment and Biomarker Utilization.
    Author: Christiansen SC, Zuraw BL.
    Journal: Immunol Allergy Clin North Am; 2024 Aug; 44(3):543-560. PubMed ID: 38937015.
    Abstract:
    The role of contact system activation has been clearly established in the pathogenesis of hereditary angioedema due to C1 inhibitor deficiency (HAE-C1INH). C1 inhibitor (C1INH)-protease complexes, levels of functional C1INH, plasma kallikrein activation, and cleavage of high-molecular-weight kininogen have each been associated with disease activity. More recently, HAE with normal levels of C1INH (HAE-nl-C1INH) has been recognized. Six genetic mutations have been identified which are linked to HAE-nl-C1INH phenotypes. The majority of individuals with HAE-nl-C1INH fall into the unknown category. There is substantial evidence that bradykinin generation underlies the recurrent attacks of swelling in some of these cohorts.
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