Fig. 2

Diagnostic work up in patients suspected to have HAE. Abbreviations: 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 factor XII (FXII) angiopoetin (ANGPTI) or plasminogen (PLG); ACEI-AE: angiotensin converting enzyme inhibitor-induced angioedema