The remaining 17% (n=57) comprised idiopathic non-histaminergic angioedema (AE-UNK; n=14), drug-induced AE due to drugs targeting the renin–angiotensin–aldosterone system (AE-DI/RAAS: AE-ACEI, AE-Sartan and AE-Gliptin; n=11), acquired angioedema due to C1INH deficiency (AAE-C1INH; n=25), and HAE with normal C1INH (HAE-FXII and HAE-PLG; n=7). Here, REN is linked to hyperinsulinemic hypoglycemia, familial, 4.