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Feature Article

Hereditary and acquired angioedemas: rare but potentially life-threatening

Constance H Katelaris

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Abstract

Angioedema without urticaria should prompt consideration of hereditary, or acquired, C1 inhibitor deficiency-associated and ACE inhibitor-induced angioedema. These rare forms require different management to urticaria-associated angioedema as they do not respond to antihistamines, corticosteroids and adrenaline.

Key Points

  • Angioedema most often occurs in association with urticaria and usually then responds to treatments used for the urticaria.
  • When angioedema occurs without urticaria, consider hereditary angioedema, acquired angioedema associated with C1 inhibitor deficiency and ACE inhibitor-induced angioedema.
  • Hereditary angioedema is rare but must be identified as it can be life-threatening. Treatments include C1 INH concentrates, icatibant and ecallantide. Danazol and tranexamic acid are used for prophylaxis.
  • Treatment of the underlying disease generally relieves symptoms in patients with the very rare acquired angioedema associated with C1 inhibitor deficiency.
  • ACE inhibitor-induced angioedema is an important cause of angioedema in older people, and may be life-threatening.

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