title: Recent advances in management and treatment of hereditary angioedema.
abstract: BACKGROUND
  Hereditary angioedema (HAE) is a rare autosomal-dominant disease characterized by recurrent self-limiting episodes of skin and mucosal edema. Morbidity and mortality are significant, and new and pending therapies are now available to reduce the risk associated with the disease.
  
  
  OBJECTIVE
  To update the reader on new advances in HAE to improve patient care.
  
  
  METHODS
  We performed a literature search of Ovid, PubMed, and Google to develop this review. Articles that are necessary for the understanding and use of the new therapeutic options for HAE were chosen, and studies of high quality were used to support the use of therapies, and in most cases, results from phase III studies were used.
  
  
  RESULTS
  Until recently, therapy for HAE attacks in the United States consisted of symptom relief with narcotics, hydration, and fresh-frozen plasma, which contains active C1 inhibitor. Therapy to prevent HAE attacks has been confined to androgens and, occasionally, antifibrinolytic agents; however, both drug groups have significant adverse effects. The approval of C1-inhibitor concentrate for prevention and acute therapy has improved efficacy and safety. Ecallantide has also been approved for therapy of attacks, and icatibant is expected to be approved in the next few months for attacks. Recombinant C1 inhibitor is presently in phase III studies and should be available for attacks in the near future.
  
  
  CONCLUSIONS
  In this article we review the changing therapeutic options available for patients in 2011 and beyond.
authors: Department of Pediatrics, Weill Cornell Medical Center, NewYork Presbyterian Hospital, New York, NY, USA.
authors: Niti
authors: N
authors: Sardana
authors: Timothy J
authors: TJ
authors: Craig
mesh_terms: D054179:Angioedemas, Hereditary / Q000188:drug therapy*; D002648:Child; D050718:Complement C1 Inhibitor Protein / Q000627:therapeutic use; D006801:Humans
chemical_list: D050718:Complement C1 Inhibitor Protein