Evidence-based recommendations for the therapeutic management of angioedema owing to hereditary C1 inhibitor deficiency: consensus report of an International Working Group

Allergy. 2012 Feb;67(2):147-57. doi: 10.1111/j.1398-9995.2011.02751.x. Epub 2011 Nov 30.

Abstract

Angioedema owing to hereditary deficiency of C1 inhibitor (HAE) is a rare, life-threatening, disabling disease. In the last 2 years, the results of well-designed and controlled trials with existing and new therapies for this condition have been published, and new treatments reached the market. Current guidelines for the treatment for HAE were released before the new trials and before the new treatments became available and were essentially based on observational studies and expert opinion. To provide evidence-based HAE treatment guidelines supported by the new studies, a conference was held in Gargnano del Garda, Italy, from September 26 to 29, 2010. The meeting hosted 58 experienced HAE expert physicians, representatives of pharmaceutical companies and representatives of HAE patients' associations. Here, we report the topics discussed during the meeting and evidence-based consensus about management approaches for HAE in adult/adolescent patients.

Publication types

  • Consensus Development Conference
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Angioedemas, Hereditary / therapy*
  • Bradykinin / administration & dosage
  • Bradykinin / adverse effects
  • Bradykinin / analogs & derivatives
  • Bradykinin / therapeutic use
  • Bradykinin Receptor Antagonists
  • Complement C1 Inactivator Proteins / deficiency*
  • Complement C1 Inhibitor Protein / administration & dosage
  • Complement C1 Inhibitor Protein / adverse effects
  • Complement C1 Inhibitor Protein / therapeutic use
  • Humans
  • Kallikreins / antagonists & inhibitors
  • Peptides / administration & dosage
  • Peptides / adverse effects
  • Peptides / therapeutic use

Substances

  • Bradykinin Receptor Antagonists
  • Complement C1 Inactivator Proteins
  • Complement C1 Inhibitor Protein
  • Peptides
  • ecallantide
  • icatibant
  • Kallikreins
  • Bradykinin