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Angioedema

Angioedema is a topic covered in the 5-Minute Clinical Consult.

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Basics

Description

  • Angioedema (AE) is acute, localized swelling of skin, mucosa, and submucosa caused by extravasation of fluid into the affected tissues.
  • AE develops in minutes to hours and resolves in hours to days but can be life-threatening if the upper airway is involved.
  • Two major classifications of AE exist:
    • Histamine- or immune-mediated AE (HIAE) with urticarial
    • Bradykinin-mediated AE without urticaria; both with unique subtypes
  • Synonym(s): angioneurotic edema; Quincke edema

Epidemiology

  • Predominant age
    • HIAE or idiopathic AE (IAE): any age
    • Hereditary AE (HAE): age of onset infancy to 2nd decade of life
    • Acquired AE (AAE): often patients >40 years
  • Predominant gender: male = female (except HAE with normal compliment: women > men)

Prevalence
  • AE occurs in 15% of the population over a lifetime.
  • AE: 0.1–2.2% of patients receiving angiotensin-converting enzyme (ACE) inhibitors: African Americans have a 4 to 5 times greater risk of ACE inhibitor–induced AE than Caucasians.
  • HAE: 1:10,000 to 50,000 population in the United States

Etiology and Pathophysiology

  • HIAE with urticarial (1):
    • Allergy to food (shellfish, nuts, eggs, milk, wheat soy), latex, venom, or medication (aspirin, NSAIDs, antibiotics, narcotics, and OCPs)
    • Acute and chronic spontaneous urticaria
    • Other urticarial/AE syndromes: cold urticaria, urticarial vasculitis, exercise-induced urticaria or anaphylaxis, episodic eosinophilia with AE, vibration-induced urticaria
    • IAE
  • Bradykinin-mediated AE without urticarial (1):
    • Attacks are triggered by prolonged mechanical pressure, cold, heat, trauma, emotional stress, menses, illness, and inflammation.
    • HAE due to C1 INH deficiency
      • Type I: the most common form, caused by decreased production of C1 esterase inhibitor (C1 INH)
      • Type II: functionally impaired C1 INH
    • HAE with normal C1 INH levels
      • Formerly type III HAE
      • Involves mutations in coagulation factor XII gene, often estrogen dependent, associated with estrogen administration
    • AAE with C1 INH deficiency
      • Rare condition
      • Associated with lymphoproliferative disorders, autoimmune diseases (e.g., lupus)
      • Type I: lack of anti–C1 INH autoantibody
      • Type II: presence of anti–C1 INH autoantibody
    • AE due to medications
      • ACE inhibitors
      • Dipeptidyl peptidase-4 inhibitors
      • Direct renin inhibitor
    • IAE

Genetics
  • HAE types I and II are autosomal dominant, whereas HAE with normal C1 INH is dominant X-linked.
  • HAE occurs in 25% of patients as a result of spontaneous genetic mutations.

Risk Factors

  • Consuming medications and foods that can cause allergic reactions
  • Preexisting diagnosis of HAE or AAE
  • Positive family history

General Prevention

  • Avoid known triggers.
  • Do not use ACE inhibitors in type I or II HAE.

Commonly Associated Conditions

  • Quincke disease (AE of the uvula)
  • Urticaria

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Citation

Stephens, Mark B., et al., editors. "Angioedema." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116031/3.4/Angioedema.
Angioedema. In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116031/3.4/Angioedema. Accessed June 27, 2019.
Angioedema. (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116031/3.4/Angioedema
Angioedema [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 June 27]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116031/3.4/Angioedema.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Angioedema ID - 116031 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116031/3.4/Angioedema PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -