Angioedema

Last Updated: 2020-11-23

Author(s): -

Quincke 1882.

Quinck edema, angioneurotic edema, angioedema, dropsy, periodic edema, atrophedema, hydrops, giant hives, oedema.

Polyaetiological, potentially life-threatening swelling of mucous membranes.

Duration

  • Usually between 24-72 hours.
  • Histamine-mediated angioedema:
    • The most common form of angioedema.
    • Causal association with urticaria.
    • No family accumulation
    • Trigger
      • Often IgE mediated.
      • Infections.
      • Mostly idiopathic.
    • Usually together with Urticaria.
  • Bradykinin-mediated angioedema:
    • Hereditary angioedema:
      • Type I:
        • Approx. 85% of all cases.
        • Autosomal dominant, mutation in the C1-INH gene (family cluster).
        • Initial manifestation usually already in childhood (5-15 years).
        • No urticaria.
        • Phenotype I.
          • C1 esterase inhibitor: quantitative disorder (↓ synthesis, ↓ activity).
          • Total complement or C4 ↓ (almost always)
      • Type II:
        • About 15% of cases.
        • Autosomal dominant, mutation in the C1-INH gene (familiy cluster).
        • C1 esterase inhibitor: qualitative disorder (↓ function)
        • No urticaria.
        • Phenotype II.
          • Non-functional C1-esterase inhibitor: normal plasma concentration.
          • Functional C1 esterase inhibitor: ↓ plasma concentration.
        • Phenotype III.
          • Non-functional C1 esterase inhibitor: ↑ plasma concentration.
          • Functional C1 esterase inhibitor: ↓ plasma concentration.
      • Type III:
        • Very rare.
        • 2 point mutations in the factor XII gene coding for the Hagemann factor (factor XII) lead to a factor XII deficiency (family cluster).
        • C1 esterase inhibitor qualitatively and quantitatively normal.
        • Normal C1 and C4.
        • No urticaria.
        • Only in women.
  • Acquired angioedema (AAE):
    • Type I: associated with lymphomas.
    • Type II: associated with lymphomas. 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. caused by aspirin
    •  Idiopathic angioedema (IAE) (diagnosis of exclusion)
    • Traumatic angioedema (see 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.

  • Medical history (ever appeared before? Family history? Trigger: Unusual medication, food intake, hymenopteran sting? Infection recall? Burning when urinating?)
  • Clinical features
  • Laboratory
  • Depending on the medical history
    • In case of suspicion of an infectious genesis: blood count, CRP, possibly ASL and ASO, urinary status.
    • In case of suspicion of food, hymenoptera or drug allergy: allergological clarification after all symptoms have disappeared.
  • C1-esterase inhibitor quantitatively and qualitatively.
  • Total IgE, RAST in atopy

Subcutaneous edema with perivascular lymphocytic infiltrate.

  • Dyspnoea.
  • Laryngeal edema.

Acute episode of hereditary angioedema:

  • Administer C1-INH purified (e.g. Berinert) 20 IU / kg slowly i.v., the injection can be repeated depending on the clinical picture.
  • The alternative is 500-2000 ml fresh plasma or "fresh frozen plasma".
  • If indicated intubation, tracheotomy or criotomy.
  • Adrenalin, glucocorticoids and antihistamines are of no use in hereditary angioedema!
  • For anaphylaxis: see Anaphylaxis
  • Analgesics for pain.

Histamine-mediated angioedema: 

  • Find 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