Table of contents
Hereditary angioedema
Guidelines
Key sources
The following summarized guidelines for the evaluation and management of hereditary angioedema are prepared by our editorial team based on guidelines from the European Academy of Allergy and Clinical Immunology (EAACI/WAO 2022) and the European Academy of Allergy and Clinical Immunology (EAACI/AAAAI 2013).
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Screening and diagnosis
Pathogenesis: as per AAAAI/EAACI 2013 guidelines, recognize that:
HAE is an autosomal dominant disease, and most patients with HAE have a positive family history of angioedema
A
HAE is caused by mutations in the serine protease inhibitor C1 resulting in a serine protease inhibitor C1 functional deficiency
B
most cases of HAE result from a deficiency of the serine protease inhibitor C1 inhibitor
B
primary mediator of swelling in patients with HAE is bradykinin.
A
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Clinical presentation
Diagnosis
Screening of family relatives
Diagnostic investigations
Blood tests
As per EAACI/WAO 2022 guidelines:
Assess blood C1 inhibitor function, C1 inhibitor protein, and C4 levels in all patients with suspected HAE.
B
Consider repeating testing for C1 inhibitor function, C1 inhibitor protein, and C4 in patients tested positive, to confirm the diagnosis of HAE-1/2.
C
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Genetic tests
Medical management
General principles: as per EAACI/WAO 2022 guidelines, ensure that all patients have an action plan.
B
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Management of acute attacks
Prophylactic treatment
Nonpharmacologic interventions
Specific circumstances
Pregnant patients: as per EAACI/WAO 2022 guidelines, prefer plasma-derived C1 inhibitors during pregnancy and lactation.
B
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Pediatric patients
Patients with normal C1 inhibitor levels
Patients with acquired angioedema
Patients with ACEI-associated angioedema