Current and Emerging Therapeutics in Hereditary Angioedema

center dot Distinguishing between mast cell -mediated versus bradykinin-mediated angioedema is paramount as effective treatment is different for these 2 classes of angioedema. center dot All patients with hereditary angioedema (HAE) should have an effective on -demand treatment which include: plasma -derived C1 esterase inhibitor (C1-INH), recombinant human C1-INH, ecallantide, or icatibant. center dot Long-term prophylaxis should be considered and personalized for all patients with HAE using current first -line options which include C1-INH concentrates, lanadlumab, or berotralstat. center dot Studies of investigational treatments to expand HAE management options are ongoing and include oral agents (kallikrein inhibitors, bradykinin B2 receptor antagonists), monoclonal antibodies targeting Factor XIIa and plasma kallikrein, RNA -targeted therapies to reduce plasma prekallikrein, and various gene therapies (clustered regularly interspaced short palindromic repeats/CRISPR-associated protein 9, adeno-associated virus [AAV]vectors, autologous hematopoietic stem cell gene therapy).