Acquired Angioedema

Overview

Type of disease: Rare conditions

Acquired angioedema (AAE) is a rare condition characterized by recurrent episodes of swelling (edema). Affected people may have swelling of the face, lips, tongue, limbs, and genitals. Sometimes there is abdominal pain due to edema of the gastrointestinal mucosa, and life-threatening edema of the upper respiratory tract. Swelling episodes may be triggered by mild trauma (e.g., dental work), viral illness, cold exposure, pregnancy, ingestion of certain foods, or emotional stress.  There are two forms of AAE: type I and type II. AAE type I is most commonly associated with B-cell lymphoproliferative disorders and consumption of C1-INH.  AAE type II is an autoimmune condition associated with the presence of an autoantibody against C1-INH. Treatment aims to control episodes and normalize C1-INH levels.  When possible, the underlying disorder should be treated.Source: Genetic and Rare Diseases Information Center (GARD), supported by ORDR-NCATS and NHGRI.

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