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Angioedema is essentially an allergic phenomenon. Treatment is common for all types of angioedema when the condition is a medical emergency.
When angioedema affects the throat and tongue and there is rapidly progressing swelling of the throat, there may be progressive narrowing of the airways leading to difficulty in breathing. The priority of management in these conditions is emergency opening up of the airways and maintenance of respiration.
If there is compromise in breathing, an endotracheal tube is inserted into the trachea and it is connected to oxygen tubes. This secures airway. Intubation should be performed as soon as possible since excessive swelling of the larynx and back of throat makes intubation difficult. In these cases a hole or opening is made over the neck (on the trachea) to enable air to pass. This is called tracheostomy.
These patients are generally advised to avoid triggers such as Angiotensin converting enzyme inhibitors and contraceptives containing estrogen.
Treatment is usually with the use of plasma-derived C1 INH concentrate. This is being used with success in Europe and Canada. Plasma-derived C1 INH concentrate however is not approved by the Food and Drug Administration for use in the United States.
Typically fresh-frozen plasma, which contains C1 INH is found to be as effective as C1 INH concentrate and symptoms improve within 30 to 60 minutes of the infusion.
Attenuated androgens including danazol are also used to increase the production of C1 INH from the liver and this prevents attacks in hereditary angioedema.