immunology allergic disorders
Hereditary and Acquired Angioedema
Treatment
For acute attacks,C1 inhibitor,ecallantide,icatibant Foracute attacks,the following are considered first-line treatment: Purified plasma-derived humanC1 inhibitor RecombinantC1 inhibitorobtained from the milk of transgenic rabbits Ecallantide(a recombinant protein that acts as a reversible inhibitor of kallikrein) Icatibant(a synthetic decapeptide that acts as a reversible, competitive antagonist of the bradykinin type 2 receptor) ( Treatment references ). If none of these drugs is available, fresh frozen plasma or, in the European Union,tranexamic acidhas been used. A recombinant form ofC1 inhibitor, recombinantC1 inhibitor, is also available. If the airways are affected, securing an airway is the highest priority.Epinephrinemay provide transient benefit in acute attacks when airways are involved. However, the benefit may not be sufficient or may be temporary; then endotracheal intubation may be necessary. Corticosteroids and antihistamines are not effective. Analgesics, antiemetics, and fluid replacement can be used to relieve symptoms. Treatment of patients with hereditary angioedema focuses on 4 core principles ( Treatment references ): Availability of effective on-demand acute therapy for all patients Early treatment to prevent attack progression Treatment of attacks regardless of the site of swelling Incorporation of long-term prophylaxis based on highly individualized decision-making reflecting a physician-patient partnership Based on these principles, all patients with confirmed hereditary angioedema should have access to ≤ 2 standard doses of an on-demand medication for treatment of acute attacks ( Treatment references ). Antihistamines and corticosteroids are not effective for hereditary or acquired angioedema. 