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The Role of Complement in Hereditary Angioedema

Marcel Levi1, Danny M Cohn2

  • 1University College London Hospitals NHS Foundation Trust, Department of Medicine, UK; Cardiometabolic Programme-NIHR UCLH/UCL BRC, London, UK; Amsterdam UMC, University of Amsterdam, Department of Vascular Medicine, Amsterdam, the Netherlands.

Transfusion Medicine Reviews
|November 3, 2019
PubMed

Insights

Hereditary angioedema (HAE) is caused by low C1 inhibitor levels, leading to attacks. New insights reveal bradykinin

Area of Science:

  • Immunology
  • Complement System
  • Plasma Proteases

Background:

  • Hereditary angioedema (HAE) is linked to low C1 inhibitor levels, causing debilitating attacks.
  • C1 inhibitor regulates complement, coagulation, and fibrinolytic systems.
  • Bradykinin overproduction, due to insufficient C1 inhibitor regulation, is key in HAE pathogenesis.

Purpose of the Study:

  • To review recent findings on HAE pathophysiology.
  • To discuss the role of bradykinin and C1 inhibitor in HAE.
  • To highlight novel therapeutic strategies for HAE.

Main Methods:

  • Review of recent scientific literature on hereditary angioedema.
  • Analysis of the role of C1 inhibitor in complement, coagulation, and fibrinolysis.
  • Examination of the kallikrein-kinin system in HAE pathogenesis.

Main Results:

  • Low C1 inhibitor levels lead to angioedema attacks.
  • Bradykinin plays a critical role in HAE pathophysiology.
  • C1 inhibitor deficiency can activate coagulation and fibrinolytic pathways.

Conclusions:

  • Understanding C1 inhibitor's role is crucial for HAE management.
  • Bradykinin pathway modulation offers new therapeutic avenues for HAE.
  • Novel treatments, including C1 inhibitor concentrates and bradykinin antagonists, are available for HAE.

Keywords:
BradykininC1 inhibitorCoagulationComplementFactor XIIHereditary angioedemaKallikreinKininPlasminogenSerine protease inhibitors

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