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):
  • 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.