Endothelial barrier disorder in hereditary angioedema: molecular mechanisms and therapeutic implications

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Hereditary angioedema (HAE) is a rare genetic disorder characterized by recurrent episodes of vascular leakage and tissue swelling.
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Endothelial dysfunction plays a crucial role in HAE pathogenesis, influencing vascular permeability beyond just excessive bradykinin.
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Recent findings suggest that endothelial cells actively regulate edema formation through alterations in intercellular junctions and the glycocalyx.
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New HAE subtypes linked to endothelial regulatory pathways indicate that endothelial dysfunction may be a primary pathogenic mechanism.
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This review proposes an integrated endothelial-centered framework for HAE, emphasizing its implications for biomarker discovery and treatment strategies.

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