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Acute treatment of edematous episodes of hereditary angioedema (HAE)

Acute treatment of edematous episodes of hereditary angioedema (HAE)
  Laryngeal attack Abdominal attack Cutaneous attack
Extremities, trunk Face, neck
C1-INH concentrate (plasma derived or recombinant) given intravenously Yes Yes Yes, unless swelling is extremely mild and not causing disability Yes
Ecallantide (United States only) Yes Yes Yes, unless extremely mild Yes
Icatibant Yes Yes Yes, unless extremely mild Yes
Plasma (solvent/detergent treated or fresh frozen) Yes, if first-line therapies are not available Yes, if first-line therapies are not available Yes, if severe and first-line therapies are not available Yes
Intubation*, transfer to ICU, rarely emergent tracheotomy Yes (consider early intubation if above agents are not available) Not applicable Not applicable May be necessary if attack spreads to involve upper airway
Wait and see (spontaneous resolution) Not sufficient Not recommended unless symptoms are mild and first-line therapies are not available Acceptable if mild Not sufficient (because angioedema can spread to involve upper airway)
All HAE attacks should be treated as early as possible, at first sign of prodromal symptoms and before the attack becomes fully established.

C1-INH: C1 inhibitor; ICU: intensive care unit.

* Consider intubation early in setting of progressive laryngeal edema or if C1-INH preparations, ecallantide, or icatibant are not available.
Modified with permission from: Bowen T, Cicardi M, Farkas H, et al. 2010 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema. Allergy, Asthma, and Clinical Immunology; 2010; 6:24. Copyright © 2010 BioMed Central Ltd.
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