Angioedema

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.

1. Przybilla B.S-2k Leitlinie: Diagnose und Therapie der Bienen und Wespengiftallergie. Allergo J 2011; 20: 318–39
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