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Angioedema Leaflet

What is angioedema?

Angioedema is a swelling of the deeper layers of the skin and/or mucous membranes that occurs at irregular intervals. Sometimes, though by no means always, triggers may be identified. Common triggers include surgical procedures, stressful situations, infections, certain foods, heat or cold. In women, hormonal changes have been implicated.
 
IMPORTANT: There are different types of angioedema! Frequently, the swelling is caused by the actions of histamine, as in the case of urticaria or allergy-related reactions. With this type of angioedema, patients may experience the sudden development of wheals, which are itching skin reactions resembling those occurring upon contact with stinging nettles. Non histamine-mediated angioedema, which is not associated with the appearance of wheals, is much less common. For this reason, diagnosis may often be significantly delayed.

In order to be able to select the appropriate treatment, it is necessary to distinguish between the different forms of angioedema. Based on the underlying mechanism, angioedema is classified as:

  • Histamine-mediated angioedema (e.g. urticaria or allergy-related, “with wheals”)
  • Bradykinin-mediated angioedema (non urticaria/allergy-related, “without wheals”)
  • Other forms of angioedema (not mediated by either histamine or bradykinin).

What are the typical symptoms of angioedema?

Swelling of the skin:

Angioedema most commonly occurs in the face, on the hand, feet, arms and legs, or the genital organs. Patients frequently report a feeling of tightness and sometimes pain.

Gastrointestinal attacks:

In certain forms of angioedema, for example hereditary angioedema (HAE), a majority of patients also experience swellings of the mucous membranes of the bowel. These patients report cramping, colicky pain lasting for days, accompanied by nausea, vomiting and diarrhoea. In some cases, these gastrointestinal complaints are the only sign of the disease.

Swelling attacks involving the airway:

Swellings that affect the head and neck (angioedema of the larynx or the tongue) may compromise the airways result in difficulty to breathe. In some cases, this can be life-threatening and can lead to asphyxiation.

How are these swelling attacks treated?

Histamine-mediated angioedema:

Swellings mediated by histamine respond well to antihistamines, corticosteroids (“cortisone”) and adrenalin (this last-named substance is used primarily in emergent treatment situations).

Bradykinin-mediated angioedema:

Angioedemas caused by the action of bradykinin are much less frequently encountered. Two treatment options are available for patients experiencing sudden attacks:

  • Icatibant, a bradykinin receptor antagonist, which is injected subcutaneously (i.e. injected into the fatty tissue underlying the deepest layer of the skin)
  • C1-INH concentrate, which is injected into a vein or administered as an intravenous infusion

Patients who undergo elective surgical procedures or dental treatments are often given medication prophylactically to prevent swelling attacks during or after the procedure.

IMPORTANT:
With very few exceptions, bradykinin-mediated forms of angioedema do NOT respond to treatment with cortisone or antihistamines.

Important for persons affected by bradykinin-mediated angioedema and their families

Especially dangerous are swelling attacks affecting the region of the larynx, which can result in acute difficulties to breathe that may progress to asphyxiation (suffocation) and death. This is an emergency. In such situations, you should summon emergency medical assistance directly from the emergency services by dialling 112, 999 or 911 or the emergency number established for your area. Patients with a known bradykinin-mediated form of angioedema should inform the emergency physician or ambulance medic of this fact so that the appropriate emergency medication can be immediately administered. It is best to always carry an emergency identification card listing the diagnosis and treatment recommendations as well as an emergency medication kit.

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