Update on bradykinin-mediated angioedema in 2020

Area of Science:

• Immunology
• Vascular Biology
• Rare Diseases

Background:

• Bradykinin-mediated angioedema (BMA) is a rare disorder characterized by vasodilation and increased vascular permeability.
• Unlike histamine-mediated angioedema, BMA lacks urticaria and does not respond to antihistamines or corticosteroids.
• Delayed diagnosis can lead to severe complications, including asphyxiation.

Purpose of the Study:

• To provide a comprehensive overview of bradykinin-mediated angioedema.
• To highlight diagnostic challenges and effective management strategies.
• To discuss the underlying pathophysiology and etiological factors.

Main Methods:

• Clinical presentation and family history assessment.
• Biological diagnostic markers including C1-inhibitor (C1-INH) levels and activity, and complement protein 4 (C4).
• Review of current and emerging therapeutic interventions.

Main Results:

• BMA results from bradykinin overproduction or impaired degradation, with hereditary or acquired causes.
• Key causes include C1 inhibitor deficiency and drug-induced reactions.
• Diagnosis relies on clinical suspicion and specific laboratory tests.

Conclusions:

• Effective acute attack management involves C1-inhibitor concentrates and icatibant.
• Long-term prophylaxis is essential, particularly before surgical or dental procedures.
• Ongoing research into novel therapies, including gene therapy, shows promise for BMA treatment.