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World Allergy Organization
WAO's mission: To be a global resource and advocate in the field of allergy, advancing excellence in clinical care through education, research and training as a world-wide alliance of allergy and clinical immunology societies.

World Allergy Congress 2007 Symposium

Angioedema


Michael A. Kaliner,
Institute for Asthma and Allergy
Wheaton, Maryland, USA

Michael A. Kaliner, MD is a former President of the AAAAI, ABAI, Allergy Section of ATS, and Head of Allergy at the NIH. He was recently recognized as the AAAAI Distinguished Clinician for 2006. Michael Kaliner was head of the Allergy Training Program at the NIH, and trained over 100 fellows in allergy and immunology. He has published 500 articles related to allergy. He is the current President of the World Allergy Organization (WAO) and has also written or participated in many of the GLORIA™ lectures.

Abstract
Taken from GLORIA Module 7: Angioedema

Authored by:
Allen P. Kaplan, Medical University of South Carolina
Charleston, South Carolina, USA

Connie H. Katelaris, Westmead Medical Centre
Westmead, NSW, Australia

Angioedema (A/O) was first described by Quincke in 1882. It is caused by the same pathophysiological factors that produce urticaria, but the reaction occurs deeper in the dermis and subcutaneous tissues. A/O is a term used to describe well-demarcated, non-pitting edema that occurs as large, erythematous, swollen areas in the subcutaneous tissues. The face, tongue, lips and eyelids are most commonly affected, but it may also involve the hands, feet, genitalia, mucous membranes and other parts of the body.

Within the spectrum of chronic idiopathic urticaria (CIU) and A/O, 50% of patients experience both urticaria and A/O, while 40% have urticarial lesions alone and 10% will have A/O and no urticaria. Typically, sporadic A/O is idiopathic; however, precipitating factors include physical factors, such as temperature changes and trauma. Other causes include IgEmediated hypersensitivity to foods, drugs, insect stings and inhalants. Non-IgE-mediated sensitivity to drugs, particularly aspirin and other nonsteroidal antiinflammatory drugs and ACE inhibitors, also occurs. Hereditary A/O is usually familial; however, acquired forms also have been associated with malignancy, in particular, lymphoproliferative disorders.

Table 1: Classification of A/O

Hereditary

Type 1: C1 esterase inhibitor deficiency
Type 2: functional abnormality of C1 esterase inhibitor


Acquired

Idiopathic (most common)

IgE-mediated (most commonly with urticaria)

Drugs
Foods
Stings
Infections (viral - Epstein Barr virus; hepatitis A, B; helminthic)

Non-IgE-mediated

Cyclooxygenase inhibition
ASA, NSAIDs
Angiotensin-converting enzyme inhibition
Systemic diseases, eg:
  Systemic lupus erythematosus
    Hypereosinophilia
    Lymphoma: Abnormal antibodies activate complement system


Physical causes

Cold
Cholinergic
Solar
  Vibratory


Other

Some contact reactions
  Autoantibodies to C1 esterase inhibitor
  (associated with malignancy, connective tissue diseases)


Acute laryngeal edema resulting from hereditary C1-esterase inhibitor deficiency or abnormality can cause life-threatening respiratory distress, leading to serious complications and fatalities for patients needing to undergo surgical procedures. Asphyxiation is the most common cause of mortality in hereditary angioedema. Time for onset of swelling to death can be dramatically short, from as little as 20 minutes, with intervals between 1-14 hours (mean, seven hours) being reported. Early symptoms reported by those affected include a tight sensation in the throat, feeling of a lump in the throat, hoarseness, dysphagia and progressive dyspnea. Mortality from HAE has significantly decreased since the advent of long-term prophylactic treatment with attenuated androgens.

This presentation will review the acute and long-term management of all forms of angioedema.

References

  1. Kaplan A. Chronic urticaria: pathogenesis and treatment. J Allergy Clin Immunol 2004; 114:465-474.
  2. Farkas H, Harmat G, Fust G, et al. Clinical management of hereditary angio-oedema in children. Pediatr Allergy Immunol 2002; 13:153-161.
  3. Nzeako U, Frigas E, Tremaine W. Hereditary angioedema: a broad review for clinicians. Arch Intern Med 2001; 161:2417-2429.
  4. Zuraw B, Herschbach J. Detection of C1-INH mutations in patients with hereditary angioedema. J Allergy Clin Immunol 2000; 105:541-546.
  5. Eck S, Morse J, Janssen D, et al. Angioedema presenting as chronic gastrointestinal symptoms. Am J Gastroenterol 1993; 88:436-439.
  6. Bork K, Barnstedt S. Treatment of 193 episodes of laryngeal edema with C1-INH concentrate in patients with hereditary angioedema. Arch Intern Med 2001; 161:714-718.
  7. Markovic S, Inwards D, Frigas E, et al. Acquired C1 esterase inhibitor deficiency. Ann Intern Med 2000; 132:144-150
  8. Brown N, Ray W, Snowden M, et al. Black Americans have an increased rate of angiotensin converting enzyme inhibitor-associated angioedema. Clin Pharmacol Ther 1996; 60:8-13.
  9. Jain M, Armstrong L, Hall J. Predisposition to and late onset of upper airway obstruction following angiotensin-converting enzyme inhibitor therapy. Chest 1992; 102:871-874.
  10. Cicardi M, Bergamaschini L, Zingale L, et al. Idiopathic nonhistaminergic angioedema. Am J Med 1999; 106:650-654.
  11. Kaplan A. Chronic Urticaria and Angioedema. N Engl J Med 2002; 346:175-179.
  12. Kaplan A, Greaves M. Angioedema. J Am Acad Dermatol 2005; 53:373-388.


Slide presentation

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