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Chapter 21. Angioedema

Kathleen A. Smith, MD; Adrienne Turner Duffield, MD

  • Localized swelling of subcutaneous and submucosal tissues secondary to increased permeability of postcapillary venules:
    • Asymmetric, nonpitting swelling of face, tongue, extremities, bowel wall
    • Laryngeal edema has high mortality (25–40%)
  • Females > males

See following table.

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Classification/Pathophysiology of Angioedema
ClassificationPathophysiologyCommon triggersTime course
Hereditary angioedema (HAE)Type I (85%)
  • C1 esterase inhibitor (C1-INH) deficiency
  • AD inheritance
  • C1-INH regulates complement, fibrinolytic, and coagulation pathways
  • Unregulated activity → release of vasoactive mediators
  • Inflammation
  • Infection
  • Minor trauma (dental procedure, intubation, local trauma from snoring)
  • Usually presents in childhood
  • Onset in hours
  • Lasts 2–4 days
Type II (15%)C1-INH present, but dysfunctional
Type III
  • Coagulation factor XII mutation
  • Estrogen dependent
  • Elevated kinin
NonhereditaryAllergicIgE mediatedType I hypersensitivity reaction → mast cell degranulation
  • Requires prior sensitization
  • NSAIDs, ASA, antibiotics, narcotics, oral contraceptives, latex, food
Onset in minutes → 1 h
Idiopathic
  • Unknown
  • Most common
Recurrent
Acquired
  • C1-INH consumption from antibody or excessive complement activation
Associated with malignancy (B-cell lymphoma, monoclonal gammopathy)
ACE inhibitor induced
  • Elevated bradykinin
  • 0.1–2.2%
  • Produces ↑ bradykinin, vasodilation
  • Weeks to years after starting drug
  • Often misdiagnosed

  • Thorough history:
    • Allergies
    • History of angioedema and known triggers
    • Current symptoms (stridor, dysphagia, dysphonia, dyspnea, abdominal pain, vomiting, diarrhea)?

Table 21-1 Suggested Prophylaxis Based on Type of Angioedema
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Table 21-1 Suggested Prophylaxis Based on Type of Angioedema
TypeProphylaxis
HAE

  1. Attenuated androgens (danazol 10 mg/kg/day, maximum 600 mg/day), 4 days before and after surgery:

    • ↑ liver production C1-INH

  2. Antifibrinolytics (ε-aminocaproic acid 1 g TID, tranexamic acid 50–75 mg/kg/day):

    • If unable to take androgens

  3. C1-INH concentrate (500–1,000 U), given 1 h before surgery, second dose available

  4. FFP (1–4 U) immediately before surgery

  5. Ideally, C1-INH levels ≥50% of normal

AllergicAvoid allergen, no medication proven helpful for prevention
IdiopathicDaily antihistamine, glucocorticoids are second line given long-term side effects

  • Regardless of etiology, have a low threshold for SECURING AIRWAY; remember ABCs
  • Avoid endotracheal intubation/LMA if possible in HAE
  • Choice of induction agent unchanged
  • Consider monitoring cuff pressures of intubated patients to identify rapid airway swelling

Table 21-2 Treatment of Angioedema Based on Type
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Table 21-2 Treatment of Angioedema Based on Type
TypeTreatment
All
  • Secure airway, surgical airway if necessary
  • Treat associated hypotension (fluids, vasopressors) and bronchospasm (beta-agonists, epinephrine)
HAE
  • Plasma-derived C1-INH (may require repeat dose)
  • FFP (second line)
  • Not responsive to epinephrine, antihistamines, or steroids
Allergic
  • Antihistamines (H1 + H2)
  • Glucocorticoids
  • Epinephrine (laryngeal edema)
Acquired
  • C1-INH concentrate, FFP
  • Treat underlying malignancy
Idiopathic
  • Glucocorticoids
ACE inhibitor induced
  • Discontinue ACE inhibitor
  • FFP, C1-INH
  • Steroids, antihistamines, epinephrine have limited effectiveness

  • Ensure adequate cuff leak prior to extubation. Do not extubate if concerned
  • Consider Cook exchange catheter when extubating
  • Monitor in ICU setting
  • If cause unknown, RAST skin testing or IgE-specific antibody testing may be indicated
    • ...

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