Current and Emerging Therapeutics in Hereditary Angioedema

Immunol Allergy Clin North Am. 2024 Aug;44(3):561-576. doi: 10.1016/j.iac.2024.03.009. Epub 2024 Apr 21.

Abstract

Angioedema is characterized by transient movement of fluid from the vasculature into the interstitial space leading to subcutaneous or submucosal non-pitting edema. Current evidence suggests that most angioedema conditions can be grouped into 2 categories: mast cell-mediated (previously termed histaminergic) or bradykinin-mediated angioedema. Although effective therapies for mast cell-mediated angioedema have existed for decades, specific therapies for bradykinin-mediated angioedema have more recently been developed. In recent years, rigorous studies of these therapies in treating hereditary angioedema (HAE) have led to regulatory approvals of medication for HAE management thereby greatly expanding HAE treatment options.

Keywords: Angioedema management/treatment; C1 inhibitor deficiency; Hereditary angioedema; Investigational therapies for angioedema; Mast cell-mediated angioedema.

Publication types

  • Review

MeSH terms

  • Angioedemas, Hereditary* / diagnosis
  • Angioedemas, Hereditary* / drug therapy
  • Angioedemas, Hereditary* / therapy
  • Animals
  • Bradykinin* / analogs & derivatives
  • Bradykinin* / metabolism
  • Complement C1 Inhibitor Protein / therapeutic use
  • Humans
  • Mast Cells / immunology
  • Mast Cells / metabolism

Substances

  • Bradykinin
  • Complement C1 Inhibitor Protein