Angioedema

Last Updated: 2022-02-04

Author(s): Anzengruber F., Navarini A.

ICD11: EB04

Quincke 1882.

Quincke's oedema, angioneurotic oedema, angioedema, AE.

Polyaetiological, potentially life-threatening swelling of the mucous membranes.

Duration

  • Mostly between 24-72 hours.
  • Histamine-mediated angioedema:
    • Most common form of angioedema.
    • Causal association with urticaria.
    • No familial clustering.
    • Trigger
      • Frequently IgE mediated.
      • Infections.
      • Mostly idiopathic.
    • Mostly co-occurrence with urticaria.
  • Bradykinin-mediated angioedema:
    • Hereditary angioedema:
      • Type I :
        • Ca. 85% of all cases.
        • Autosomal dominant, mutation in the C1-INH gene (familial cluster).
        • First manifestation usually in childhood (5-15 yrs).
        • No urticaria.
        • Phenotype I:
          • C1 esterase inhibitor: quantitative disorder (↓ synthesis, ↓ activity).
          • Total complement or C4 ↓ (almost always).
      • Type II:
        • Ca. 15% of cases.
        • Autosomal dominant, mutation in the C1-INH gene (familial cluster).
        • C1 esterase inhibitor: qualitative disorder (↓ function).
        • No urticaria.
        • Phenotype II
          • Non-functional C1 esterase inhibitor: normal concentration in plasma.
          • Functional C1 esterase inhibitor: ↓ concentration in plasma.
        • Phenotype III
          • Non-functional C1 esterase inhibitor: ↑ concentration in plasma.
          • Functional C1 esterase inhibitor: ↓ concentration in plasma.
      • Type III:
        • Very rare.
        • 2 point mutations in the factor XII gene that codes for Hagemann factor (factor XII) lead to factor XII deficiency (familial cluster).
        • C1 esterase inhibitor qualitatively and quantitatively normal.
        • Normative C1 and C4.
        • No urticaria.
        • Only in females.
  • Acquired angioedema (AAE):
    • Type I: associated with lymphoma.
    • Type II: associated with lymphoma. Antibodies against C1-INH.
  • Renin-aldosterone angiotensin system blocker-induced angioedema (RAE):
    • Trigger:
      • ACE inhibitors.
      • Angiotensin II receptor antagonists.
  • Other forms:
    • Pseudoallergic angioedema (PAE) e.g. due to aspirin
    • Idiopathic angioedema (IAE) (diagnosis of exclusion)
    • Traumatic angioedema (see below Angioedema, vibratory)
    • Angioedema, episodic with eosinophilia.

Chronic swelling of the mucous membranes (face (eyelids, lips, tongue), genital area, respiratory tract, GI tract), but also hands and feet possible.

  • Anamnesis (ever occurred before? Family history? Trigger: unusual intake of medication, food, hymenopteran sting? Infection remembered? Burning during urination?)
  • Clinic
  • Laboratory
  • Depending on medical history
    • If infectious etiology is suspected: blood count, CRP, ASL and ASO if applicable, U status.
    • If food, hymenopteran or drug allergy is suspeced: allergological clarification after resolution of all symptoms.
  • C1 esterase inhibitor quantitatively and qualitatively.
  • IgE, RAST (Radio-Allergo-Sorbent-Test) in atopy

Subcutaneous oedema with perivascular lymphocytic infiltrate.

  • Dyspnoea.
  • Laryngeal oedema.

Acute episode of hereditary angioedema:

  • Give C1-INH purified (e.g. Berinert) 20 IU / kg slowly i.v., the injection can be repeated depending on the clinical picture.
  • Alternative is 500-2000 ml fresh plasma or "fresh frozen plasma".
  • If indicated intubation, tracheostomy or coniotomy.
  • Adrenaline, glucocorticoids and antihistamines are of no use in hereditary angioedema!
  • In the case of anaphylaxis: see anaphylaxis
  • Analgesics for pain

Histamine-mediated angioedema:

  • Look for the cause, avoid it.
  • See Therapy of chronic urticaria: prednisone, antihistamines.
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  2. Bingham, C. (2016). New-onset urticaria. Uptodate.com. Retrieved 31 May 2016, from http://www.uptodate.com/contents/new-onset-urticaria?source=search_result&search=acute+urticaria&selectedTitle=1~23
  3. Cicardi, M. (2016). Hereditary angioedema: Treatment of acute attacks. Uptodate.com. Retrieved 31 May 2016, from http://www.uptodate.com/contents/hereditary-angioedema-treatment-of-acute-attacks?source=search_result&search=heredit%C3%A4ren+angio%C3%B6dems&selectedTitle=2~142
  4. medStandards Notfall-Standards Algorithmen. (2016). Medstandards.ch. Retrieved 31 May 2016, from http://www.medstandards.ch/notfallstandards/anaphylaxie.php