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Table 4 Classification of angioedema

From: The international WAO/EAACI guideline for the management of hereditary angioedema – the 2017 revision and update

Bradykinin-induced AE Mast Cell Mediator induced AE Unknown mediator
C1-INH deficiency/defect C1-INH normal IgE mediated non-IgE mediated  
Inherited Acquired Inherited Acquired    
HAE-1
HAE-2
AAE-C1-INH HAE nC1-INH (HAE-FXII, HAE-ANGPTI, HAE-PLG, HAE-UNK) ACEI-AE Angioedema with AnaphylaxisUrticaria Angioedema with urticaria Idiopathic AE
  1. HAE-1 hereditary angioedema due to C1-Inhibitor deficiency, HAE-2 hereditary angioedema due to C1-Inhibitor dysfunction, AAE-C1-INH acquired angioedema due to C1-Inhibitor deficiency, HAE nC1-INH hereditary angioedema with normal C1-Inhibitor levels, either due to a mutation in FXII, ANGPTI, PLG or unknown (HAE-FXII, HAE-ANGPTI, HAE-PLG, HAE-UNK), ACEI-AE angiotensin converting enzyme inhibitor-induced angioedema