Quincke’s Edema: Drug Therapy

Therapy goals

• Decrease of the symptomatology
• “Long-term prophylaxis should reduce the burden of the disease by preventing or relieving attacks” [HAE guideline: see below].

Therapy recommendations

• Patients with edema of the head should be hospitalized immediately because of the risk of suffocation. [In severe cases, airway protection is urgently required.]
• Review of permanent medication due topossible effect on the existing disease.
• Acute therapy of histamine-mediated Quincke’s edema/angioedema (proceed according to scheme as for allergic reactions):
  • Epinephrine, 0.1 mg (sympathomimetic).
  • Dimetindene (H1 antihistamine): 4-8 mg i.v.; cimetidine (H2 antihistamine), 200-600 mg i.v.
  • Prednisolone equivalent,250-500 mg i.v. (glucocorticoids).
  • Salbutamol (β-symphaticomimetic).
• Note: If the angioedema does not respond to antihistamines or glucocorticoids, it could be bradykinin-mediated fluid accumulation (→ off-label use with C1 inhibitor concentrate or bradykinin B2 receptor antagonist (see below).
• Bradykinin-mediated angioedema.
  • Therapy of C1 esterase inhibitor deficiency (hereditary angioedema (HAE) with C1-INH deficiency or acquired C1 esterase deficiency* ):
    • Acute therapy:
      • C1 inhibitor concentrate [for HAE].
      • Icatibant (bradykinin B2 receptor antagonist) [for HAE and acquired C1 esterase deficiency; may be prescribed as stand-by/emergency therapy if necessary].
    • Long-term prophylaxis (LTP): danazol (androgen derivatives), tranexamic acid (antifibrinolytics); severe cases only: C1 inhibitor concentrate; furthermore: Icatibant (bradykinin B2 receptor antagonist), kallikrein inhibitors (ecallantide; lanadelumab (monoclonal antibody))* In the case of acquired C1 esterase deficiency, therapy of the underlying paraneoplastic/autoimmune disease is essential.
  • Drug-induced
    • Antihistamine/glucocorticoid; C1 inhibitor concentrate (i.v.); Icatibant (bradykinin B2 receptor antagonist) [off-label therapy].
• Basically, the triggering agent should be removed as soon as possible!

Notes for HAE patients:

• Every HAE patient should always carry sufficient on-demand medication to treat two attacks.
• Patients and their families should learn injection techniques to enable them to self-care.