[Pathophysiology and advances in the treatment of Hereditary Angioedema].

Hereditary angioedema is an autosomic dominant disease, caracterized by recurrent angioedema and caused by a defective enzime known as C1 inhibitor. The main mediator involved in the development of angioedema is bradikynin. There are three types of the disease, the first one related to a decrease in the production of the enzime, the second one caracterized by an altered function of the enzime and the third one due to an altered factor XII gene. The recurrent angioedema may be localized in the periphery, in the gastrointestinal system or laryngeal, the last one being life threatening. For its treatment there are a lot of drugs available, such as attenuated androgens, fresh frozen plasma, tranexamic acid or the enzime substitution by a C1 inhibitor concentrate purified from plasma, nanofiltered or recombinant. Recently, treatment has been directed toward the avoidance of baradykinin production or its action through its B2 receptor.