TABLE 1.

Summary of Key Criteria for the Diagnosis of HAE for Published Guidance

Category	US HAEA Guidelines8	Brazilian Guidelines9	WAO/EAACI Guidelines6	International Consensus Guidelines for Pediatric Patients10	International Consensus Algorithm11	International/ Canadian HAE Guideline12
Primary criteria	Recurrent cutaneous angioedema (without urticaria)Abdominal symptomsOropharyngeal/laryngeal swelling	Noninflammatory subcutaneous angioedema lasting longer than 12 hAbdominal pain of undefined organic etiology lasting longer than 6 hRecurrent laryngeal edema	History of recurrent angioedema attacks	Pediatric patient with angioedema of unknown etiologyFamily history	Recurrent angioedema (without urticaria)Recurrent episodes of abdominal pain and vomitingLaryngeal edemaFamily history of angioedema	Recurrent angioedema (without urticaria)Recurrent abdominal pain/swelling
Secondary criteria	Screening of first-degree relatives	Family history of angioedema	Family history of HAEOnset of symptoms in childhood or adolescenceRecurrent and painful abdominal symptomsOccurrence of upper airway edemaFailure to respond to antihistamines, glucocorticoids, or epinephrinePresence of prodromal signs or symptoms before swellingsAbsence of urticaria			No response to allergy treatments
Laboratory tests	C4 levelC1-INH functional and antigenic level	Quantitative C1-INH, <50% in 2 distinct samplesFunctional C1-INH, <50% in 2 distinct samplesMutation in SERPING1	C4 levelC1-INH functional and antigenic level	C4 levelC1-INH functional and antigenic level	C4 levelC1-INH functional and antigenic level	C4 levelC1-INH functional and antigenic level

C1-INH indicates C1 inhibitor; EAACI, European Academy of Allergy and Clinical Immunology; HAE, hereditary angioedema; US HAEA, United States Hereditary Angioedema Association; WAO, World Allergy Organization.