Medical algorithm: Management of C1 inhibitor hereditary angioedema

C1-inhibitor (C1INH) inhibits several proteins from the complement, coagulation, kallikrein-kinin and fibrinolysis systems [1,2]. The decrease in functional C1INH (fC1INH) produces an excess of bradykinin and local increase in vascular permeability, resulting in angioedema (AE) [2,3]. The genetic deficiency of C1INH is responsible for two types of hereditary angioedema (HAE): HAE due to a deficiency in C1INH levels (HAE-C1INH-1) and HAE due to a dysfunctional C1INH with normal or elevated C1INH levels (HAE-C1INH-2) [4,5,6].