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Hereditary angioedema

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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Guidelines

1.Screening and diagnosis

Pathogenesis: 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

2.Diagnostic investigations

Blood tests
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

3.Medical management

General principles: ensure that all patients have an action plan.
B
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  • Management of acute attacks

  • Prophylactic treatment

4.Nonpharmacologic interventions

Avoidance of triggers: as per WAO 2022 guidelines, educate all patients about triggers likely to induce attacks.
B

5.Specific circumstances

Pregnant patients: as per 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