title: Recent advances in the management of hereditary angioedema.
abstract: Hereditary angioedema (HAE) is a rare genetic condition that manifests as painful and potentially life-threatening episodic attacks of cutaneous and submucosal swelling. It results from functional deficiency of C1 inhibitor (C1 INH), which is a regulator of the complement, fibrinolytic, kinin (contact), and coagulation systems. In patients with HAE, the low plasma concentration of functional C1 INH leads to overactivation of the kinin cascade and local release of bradykinin. Bradykinin is responsible for the pain, vascular permeability changes, and edema associated with HAE. Until recently, therapeutic options for HAE have been very limited. Many new therapies have emerged, however, such as C1 INH replacement drugs and medications aimed at components of the contact system (eg, plasma kallikrein inhibitor and bradykinin B2 receptor antagonist). The authors review current and novel treatments for patients with HAE.
authors.affiliation: Department of Medicine, Lehigh Valley Health Network, Allentown, Pennsylvania, USA.
authors.forename: Stephen E
authors.initials: SE
authors.lastname: Hemperly
authors.forename: Niti Sardana
authors.initials: NS
authors.lastname: Agarwal
authors.forename: Ying-Yang
authors.initials: YY
authors.lastname: Xu
authors.forename: Yu-Xiang
authors.initials: YX
authors.lastname: Zhi
authors.forename: Timothy J
authors.initials: TJ
authors.lastname: Craig
mesh_terms: D000538:Aluminum Silicates; D054179:Angioedemas, Hereditary / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology / Q000503:physiopathology / Q000517:prevention & control; D002196:Capillaries; D003174:Complement C1 Inactivator Proteins / Q000009:adverse effects / Q000627:therapeutic use*; D050718:Complement C1 Inhibitor Protein / Q000627:therapeutic use*; D003937:Diagnosis, Differential; D006801:Humans; D007610:Kallikreins / Q000037:antagonists & inhibitors; D010455:Peptides / Q000627:therapeutic use
chemical_list: D000538:Aluminum Silicates; D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; D010455:Peptides; C469952:SERPING1 protein, human; C436844:beringite; C511194:ecallantide; D007610:Kallikreins