title: US Hereditary Angioedema Association Medical Advisory Board 2013 recommendations for the management of hereditary angioedema due to C1 inhibitor deficiency.
abstract: BACKGROUND
  The treatment of hereditary angioedema (HAE) has undergone dramatic changes as newer medicines have become available in recent years. Optimal care of these patients requires a comprehensive management plan. Although several consensus papers have been published concerning the diagnosis and treatment of HAE, guidelines for a comprehensive management plan have not been developed.
  
  
  OBJECTIVE
  To develop state-of-the-art recommendations for the treatment and management of HAE due to C1 inhibitor (C1INH) deficiency in the United States.
  
  
  METHODS
  Members of the US Hereditary Angioedema Association Medical Advisory Board began by reviewing the literature concerning treatment of HAE. Preliminary recommendations were developed based on the literature review, discussions in a face-to-face meeting, and refinements in a series of drafts. Final recommendations reflect the unanimous consensus of the medical advisory board and the US Hereditary Angioedema Association leadership.
  
  
  RESULTS
  Recommendations are provided regarding a comprehensive care plan for HAE, including the following: development of an overall management plan, treatment of angioedema attacks, prophylactic treatment, and patient monitoring.
  
  
  CONCLUSIONS
  A comprehensive individualized management plan developed between an expert HAE physician and the patient, in collaboration with local medical providers and emergency departments, can provide patients with the best opportunity to lead a normal life.
authors.affiliation: Division of Rheumatology, Allergy and Immunology, Department of Medicine, University of California San Diego, La Jolla, Calif; Medicine Service, San Diego Veterans Administration Healthcare, San Diego, Calif. Electronic address: bzuraw@ucsd.edu.
authors.forename: Bruce L
authors.initials: BL
authors.lastname: Zuraw
authors.affiliation: Division of Allergy and Immunology, Department of Medicine, Harvard Medical School, Boston, Mass.
authors.forename: Aleena
authors.initials: A
authors.lastname: Banerji
authors.affiliation: Division of Immunology/Allergy, Department of Internal Medicine, University of Cincinnati College of Medicine, Cincinnati, Ohio.
authors.forename: Jonathan A
authors.initials: JA
authors.lastname: Bernstein
authors.affiliation: Division of Clinical Immunology, Department of Medicine, Mount Sinai School of Medicine, New York, NY.
authors.forename: Paula J
authors.initials: PJ
authors.lastname: Busse
authors.affiliation: Division of Rheumatology, Allergy and Immunology, Department of Medicine, University of California San Diego, La Jolla, Calif; Allergy Department, Southern California Kaiser Permanente, San Diego, Calif.
authors.forename: Sandra C
authors.initials: SC
authors.lastname: Christiansen
authors.affiliation: Division of Rheumatology & Immunology, Winthrop University Hospital, Mineola, NY.
authors.forename: Mark
authors.initials: M
authors.lastname: Davis-Lorton
authors.affiliation: Division of Pediatric Allergy and Immunology, Department of Pediatrics, Duke University Medical Center, Durham, NC.
authors.forename: Michael M
authors.initials: MM
authors.lastname: Frank
authors.affiliation: Institute for Asthma and Allergy, Chevy Chase, Md.
authors.forename: Henry H
authors.initials: HH
authors.lastname: Li
authors.affiliation: Allergy and Asthma Specialists, Dallas, Tex.
authors.forename: William R
authors.initials: WR
authors.lastname: Lumry
authors.affiliation: Clinical Immunology and Allergy, Department of Medicine, University of California Los Angeles, Los Angeles, Calif.
authors.forename: Marc
authors.initials: M
authors.lastname: Riedl
mesh_terms: D054179:Angioedemas, Hereditary / Q000517:prevention & control / Q000628:therapy*; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency; D050718:Complement C1 Inhibitor Protein; D006801:Humans; D014481:United States
chemical_list: D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; C469952:SERPING1 protein, human
keywords: Bradykinin; C1 inhibitor; Hereditary angioedema; Management; On-demand treatment; Prophylactic treatment