Hereditary angioedema (HAE) owing to C1 esterase inhibitor (C1-INH) deficiency (HAE-C1-INH), HAE with normal C1-INH, and acquired angioedema due to C1-INH deficiency are rare but serious disorders with high morbidity and mortality. The body of knowledge on the pathophysiology of angioedema has grown significantly, resulting in new clinical studies using innovative treatment drugs and techniques. HAE-C1-INH management strategies attempt to cure acute episodes or prevent them through preventive medication. Plasma-derived C1-INH concentrates, a bradykinin B2 receptor (B2R) antagonist, and recombinant human C1-INH are all available in Europe to treat acute attacks. A plasma-derived C1-INH concentrate, a bradykinin B2R antagonist, and a plasma kallikrein inhibitor are all licensed in the United States for the treatment of acute HAE-C1-INH attacks. Short-term prophylactic therapy includes C1-INH concentrates and attenuated androgens. Attenuated androgens, a plasma-derived C1-INH concentrate, and antifibrinolytics are long-term preventive therapies. Self-administration of plasma-derived C1-INH and a bradykinin B2R antagonist is permitted at home.


The number of treatment options for HAE-C1-INH and comparable illnesses has grown significantly in recent years, assisting in the reduction of the burden of these uncommon diseases.