ANGIOEDEMA DUE TO THE LACK OF C1- INIBITOR :

what it is, what the symptoms are and how to treat it.

The deficiency of the inhibitor of the first component of the Complement System, C1-inibitor (C 1-INH), can be caused by a genetic defect (hereditary angioedema) or by an increased consumption (acquired angioedema).

Symptoms are recurring swellings lasting 2 to 5 days. When the skin is involved, the affected area appears swollen, pale, not pitting and usually without a clear demarcation between affected and healthy parts.

In the case of mucous involvement particularly dangerous is the glottis edema that can cause death for asphyxia. Frequently it can also occur in the bowel causing abdominal colic very similar to an "acute appendicitis".

It is not a disease caused by allergies and therefore can not be treated with anti allergic drugs. Trauma even minimal, and psychological stress can facilitate attacks. For the same reason operations in the mouth, such as dental surgery, endoscopic examinations, etc., can cause edema of the glottis.

The diagnosis of the disease is relatively simple and consists on the quantitative and functional determination of C1-INH.

The correct therapy, in case of an attack of the glottis, is the infusion of an adequate amount of C1-INH plasma concentrate or, especially in the early stages of the attack, the synthetic product Firazyr ® (Icatibant / acetate), an inhibitor of bradykinin, available in Italy. Prevention of attacks, in adult people, is based on chronic administration of attenuated androgens. The administration of cortisone and antihistamines is useless.

BIBLIOGRAPHY : Agostoni A. - Cicardi M. Medicine - Baltimore71 :.206-215, 1992