Laryngeal Edema

  • Alla Volokha


Hereditary angioedema (HAE) is characterized by recurrent episodes of angioedema, without urticaria or pruritus

HAE often affects the skin and mucosal tissues of the upper respiratory and gastrointestinal tracts

Laryngeal attack is a common life-threatening complication and may cause fatal asphyxiation

Worldwide incidence of HAE estimated from 1:10,000 to 1:50,000

HAE has an autosomal dominant inheritance, while up to 20–25% patients may have new spontaneous mutations

Decrease activity or absence of C1-INH results leads to excessive bradykinin production during angioedema attacks

C4 and C1-INH level and function, are the key laboratory values in the diagnosis of hereditary angioedema

Patients with hereditary angioedema have poor response to treatment with antihistamines and steroids

First-line therapies for HAE are human plasma-derived C1 inhibitor concentrate or recombinant human C1 inhibitor, which are available only in the European Union, or Icatibant, a bradykinin B2 receptor antagonist


Hereditary angioedema Icatibant C1 esterase inhibitor Bradykinin 


  1. 1.
    Bowen T. Hereditary angioedema: beyond international consensus - circa December 2010 - The Canadian Society of Allergy and Clinical Immunology Dr. David McCourtie Lecture. Allergy Asthma Clin Immunol. 2011;7(1):1.CrossRefGoogle Scholar
  2. 2.
    Bowen T, Cicardi M, Farkas H, Bork K, Longhurst HJ, Zuraw B, Aygoeren-Pürsün E, Craig T, Binkley K, Hebert J, Ritchie B, Bouillet L, Betschel S, Cogar D, Dean J, Devaraj R, Hamed A, Kamra P, Keith PK, Lacuesta G, Leith E, Lyons H, Mace S, Mako B, Neurath D, Poon MC, Rivard GE, Schellenberg R, Rowan D, Rowe A, Stark D, Sur S, Tsai E, Warrington R, Waserman S, Ameratunga R, Bernstein J, Björkander J, Brosz K, Brosz J, Bygum A, Caballero T, Frank M, Fust G, Harmat G, Kanani A, Kreuz W, Levi M, Li H, Martinez-Saguer I, Moldovan D, Nagy I, Nielsen EW, Nordenfelt P, Reshef A, Rusicke E, Smith-Foltz S, Späth P, Varga L, Xiang ZY. 2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema. Allergy Asthma Clin Immunol. 2010;6(1):24.CrossRefGoogle Scholar
  3. 3.
    Bork K, Meng G, Staubach P, Hardt J. Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am J Med. 2006;119(3):267–74.CrossRefGoogle Scholar
  4. 4.
    Craig T, Aygoren-Pursun E, Bork K, Bowen T, Boysen H, Farkas H, Grumach A, Katelaris CH, Lockey R, Longhurst H, Lumry W, Magerl M, Martinez-Saguer I, Ritchie B, Nast A, Pawankar R, Zuraw B, Maurer MWAO. Guideline for the management of hereditary angioedema. World Allergy Organ J. 2012;5(12):182–99.CrossRefGoogle Scholar
  5. 5.
    Farkas H, Varga L, Szeplaki G, Visy B, Harmat G, Bowen T. Management of hereditary angioedema in pediatric patients. Pediatrics. 2007;120(3):e713–22.CrossRefGoogle Scholar
  6. 6.
    Wahn V, Aberer W, Eberl W, Fasshauer M, Kuhne T, Kurnik K, Magerl M, Meyer-Olson D, Martinez-Saguer I, Spath P, Staubach-Renz P, Kreuz W. Hereditary angioedema (HAE) in children and adolescents--a consensus on therapeutic strategies. Eur J Pediatr. 2012;171(9):1339–48.CrossRefGoogle Scholar
  7. 7.
    Ebo DG, Verweij MM, De Knop KJ, Hagendorens MM, Bridts CH, De Clerck LS, Stevens WJ. Hereditary angioedema in childhood: an approach to management. Paediatr Drugs. 2010;12(4):257–68.CrossRefGoogle Scholar
  8. 8.
    Bork K, Barnstedt SE. Treatment of 193 episodes of laryngeal edema with C1 inhibitor concentrate in patients with hereditary angioedema. Arch Intern Med. 2001;161(5):714–8.CrossRefGoogle Scholar
  9. 9.
    Farkas H, Jakab L, Temesszentandrasi G, Visy B, Harmat G, Fust G, Szeplaki G, Fekete B, Karadi I, Varga L. Hereditary angioedema: a decade of human C1-inhibitor concentrate therapy. J Allergy Clin Immunol. 2007;120(4):941–7.CrossRefGoogle Scholar
  10. 10.
    Grigoriadou S, Longhurst HJ. Clinical immunology review series: an approach to the patient with angio-oedema. Clin Exp Immunol. 2009;155(3):367–77.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Alla Volokha
    • 1
    • 2
  1. 1.Shupyk National Medical Academy for Postgraduate Education, Department of Pediatric Infectious Diseases and Pediatric ImmunologyKievUkraine
  2. 2.Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN)KievUkraine

Personalised recommendations