Angioedema of the head and neck regionT78.3, T78.4, D84.-, T88.7

Angioedema of the upper airway is a potential life-threatening emergency. For clinical practice, a rough classification according to their etiopathogenesis has proven useful.

Classification of angioedema of the head and neck region (varied n. Bas et al. 2013)

• Histamine-mediated angioedema (1%!)
• Non-histamine-mediated angioedema
  • acute phase-mediated angioedema
    • inflammation-induced angioedema - due to e.g. peritonsillar abscess or acute tonsillitis with accompanying inflammatory reactions (about 79%)
    • tumor-induced angioedema (7%)
  • bradykinin-induced angioedema (8%)
  • idiopathic angioedema (5%)

Acute-phase edema: Inflammatory edema of the head and neck region represents the most frequent events in a general consultation, accounting for about 80% of all acute edema in this region. Causes are infectious-inflammatory diseases such as peritonsillar abscesses or acute tonsillitis with accompanying inflammatory edema. Painful, usually unilateral protrusion of the soft palate with edema of the uvula occurs.

Bradykinin-induced acquired angioedema: At 8%, these represent the second most common group of acute edemas of the head and neck region. They affect about 30,000 people/year in Germany. Bradykinin-induced angioedema is caused in the vast majority of cases by the use of ACE inhibitors. More rarely, angiotensin type I receptor blockers (sartans) are causative. Bradykinin-induced angioedema occurs predominantly in the first 3 years of use. However, there are also cases in which much longer latency periods (up to 11 years) have been observed.

Histamine-mediated acquired angioedema: quite predominantly in patients with acute or chronic urticaria. Here, the clinical presentation quickly leads to the diagnosis. In classic type I reactions, the relationship between exposure and clinical reaction is usually evident and also remembered by the patient.

Other acquired angioedema (AAE)

Hereditary angioedema (HAE/s.u. Angioedema hereditary; HAE typeI - HAE type VIII)

Diagnostic pathway for acute angioedema of the head and neck region

• Alerting of an airway team (anesthesiologist, emergency physician)
• Securing the airway (oral pharyngeal examination)
• Where is the angioedema located? (tongue, soft palate, hypopharynx/larynx, face-lip)

Therapy of histamine-induced anigoedema

• Clemastine 2 mg i.v.
• Prednisolone 500 mg i.v.
• If necessary, adrenaline in case of circulatory involvement (0.3-0.5 mg adrenaline i.m.)

Therapy of bradykinin-induced angioedema

• Icatibant 30 mg s.c. (effect after 30 to 45 min)
• alternatively -at present still experimental-: C1-inhibitor concentrate (Berinert P, Cinryze, Ruconest) or administration of a kallikrein inhibitor (Kalbitor)
• if necessary adrenaline in case of circulatory involvement (0.3-0.5mg adrenaline i.m.)

1. Bas M et al (2013) Angioedema of the head and neck region. Allergo J 22:118-123
2. Firazyr. [http://ec.europa.eu/health/documents/community-register/2018/20180426140571/anx_140571_en.pdf EMA SUMMARY OF THE CHARACTERISTICS OF THE MEDICINE]