Management of Hereditary Angioedema
Summary
Hereditary angioedema (HAE) is a rare genetic disorder characterised by recurrent, non-itchy swelling of the skin, mucous membranes and submucosal tissues, with potentially life-threatening airway involvement. The underlying defect involves insufficient functional C1 inhibitor (C1-INH), which leads to uncontrolled activation of the plasma contact system, excessive generation of bradykinin and increased vascular permeability. Clinical management is divided into acute attack therapy and prophylaxis. Acute interventions aim to reverse swelling rapidly by replacing C1-INH or blocking bradykinin formation or action, using agents such as plasma-derived or recombinant C1-INH, bradykinin receptor antagonists and kallikrein inhibitors. Preventive strategies include long-term prophylaxis with regular C1-INH infusions, monoclonal antibodies targeting kallikrein, oral kallikrein inhibitors, attenuated androgens and antifibrinolytics, as well as short-term measures before surgery or major stress. Patient education, self-administration programmes and personalised treatment plans are central to reducing morbidity, improving quality of life and preventing fatal laryngeal oedema. Special considerations apply to children, pregnant or breastfeeding women and individuals with normal C1-INH variants. Multidisciplinary collaboration and structured emergency plans underpin safe home-based management and timely access to specialist care, while global efforts strive to harmonise standards and address disparities in treatment availability.
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Management of Hereditary Angioedema publication trend
The graph below shows the total number of articles in management of hereditary angioedema across all publications each year (not limited to Nature Index journals).
Technical terms
C1 inhibitor (C1-INH): a plasma serine protease inhibitor that regulates complement and contact pathways; its deficiency is central to HAE pathogenesis.
Bradykinin: a potent vasoactive peptide generated by kallikrein that increases vascular permeability and mediates the swelling in HAE.
On-demand therapy: treatment administered at the onset of an acute angioedema attack to abort or mitigate symptoms rapidly.
Prophylaxis: preventive treatment to reduce the frequency or severity of HAE attacks, classified as short-term (before known triggers) or long-term (regular administration).
Kallikrein: a serine protease in the contact system that cleaves high-molecular-weight kininogen to release bradykinin; a key therapeutic target.
References
- The international WAO/EAACI guideline for the management of hereditary angioedema—The 2021 revision and update. Allergy (2022).
- The International/Canadian Hereditary Angioedema Guideline. Allergy, Asthma & Clinical Immunology (2019).
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