Acute Management of Hereditary Angioedema Attacks

Immunol Allergy Clin North Am. 2017 Aug;37(3):541-556. doi: 10.1016/j.iac.2017.04.002. Epub 2017 May 23.

Abstract

Several treatment modalities have become available for management of acute hereditary angioedema (HAE) attacks in the last 15 years. Most are now available to patients in North America, Europe, United Kingdom, and Australia, but few options exist in developing countries. Preferred contemporary use of the treatments to be discussed is "on demand," because control remains with the patient and delays in treatment access avoided. Four treatments-plasma-derived C1 inhibitor concentrate, recombinant C1 inhibitor concentrate, ecallantide, and icatibant-are reviewed in this article. All have been shown to be superior to placebo and effective in the management of all HAE attacks.

Keywords: C1-inhibitor concentrate; Ecallantide; HAE acute attacks; Hereditary angioedema; Icatibant; Treatment of HAE; rh C1-INH.

Publication types

  • Review

MeSH terms

  • Acute Disease
  • Angioedemas, Hereditary / diagnosis*
  • Angioedemas, Hereditary / therapy*
  • Complement C1 Inhibitor Protein / administration & dosage
  • Complement C1 Inhibitor Protein / adverse effects
  • Complement C1 Inhibitor Protein / therapeutic use
  • Disease Management
  • Disease Progression
  • Humans
  • Recombinant Proteins / administration & dosage
  • Recombinant Proteins / adverse effects
  • Recombinant Proteins / therapeutic use
  • Recurrence
  • Treatment Outcome

Substances

  • Complement C1 Inhibitor Protein
  • Recombinant Proteins
  • SERPING1 protein, human
  • conestat alfa