Vnitr Lek 2026, 72(6):396-400 | DOI: 10.36290/vnl.2026.073

Modern treatment of hereditary angioedema due to C1 inhibitor deficiency: from attack control to complete disease control

Roman Hakl, Jan Baroš, Jiří Litzman
Ústav klinické imunologie a alergologie, FN u sv. Anny v Brně, Lékařská fakulta Masarykovy univerzity v Brně

Hereditary angioedema due to C1 inhibitor deficiency (HAE-C1-INH) is a rare, potentially life-threatening disorder characterized by recurrent episodes of bradykinin-mediated angioedema. In recent years, major advances have been achieved in both the diagnosis and treatment of HAE-C1-INH, particularly due to the development of targeted biological and molecular therapies. The updated 2025 WAO guidelines emphasize an individualized approach, the availability of effective on-demand therapy for all patients, and a treatment strategy aimed at achieving complete disease control. This review article summarizes current therapeutic options for HAE-C1-INH, including on-demand treatment of acute attacks, short-term prophylaxis, and long-term prophylaxis. Both established therapeutic approaches (C1 inhibitor concentrates, bradykinin B2 receptor antagonists, and kallikrein inhibitors) and novel treatment strategies, including oral kallikrein inhibitors, monoclonal antibodies, and antisense oligonucleotides, are discussed.

Keywords: hereditary angioedema, C1 inhibitor, bradykinin, kallikrein, prophylaxis, biological therapy.

Accepted: September 15, 2026; Published: October 2, 2026  Show citation

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Hakl R, Baroš J, Litzman J. Modern treatment of hereditary angioedema due to C1 inhibitor deficiency: from attack control to complete disease control. Vnitr Lek. 2026;72(6):396-400. doi: 10.36290/vnl.2026.073.
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