Fig. 1.

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 ANGPT1 (angiopoietin1), PLG (plasminogen), KNG1 (kininogen), MYOF (myoferlin), and HS3ST6 (heparan sulfate-glucosamine 3-O-sulfotransferase 6) or unknown (UNK). ACEi-AE angiotensin converting enzyme inhibitor-induced angioedema, ∗ other drugs like angiotensin II receptor blockers, gliptins, neprilysin inhibitors or tissue plasminogen activators might induce bradykinin-mediated