Efficacy of C1Inhibitor concentrate in hereditary angioedema with C1Inhibitor deficiency: analysis in the French Cohort

Hereditary angioedema (HAE) is a rare genetic disease characterized by transitory recurrent subcutaneous and/or submucosal swelling episodes, which mainly affect skin, gastrointestinal tract and upper airways1. It should be divided into 2 forms according to the level of C1 inhibitor: HAE with C1 inhibitor deficiency (C1INH-HAE)1, and with a normal C1-Inhibitor level2, C1INH-HAE being the most frequent1. C1INH-HAE can be life-threatening especially when it affects upper airways3.

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