Acquired angioedema

December 31, 2014

Synonyms

Overview

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.

Symptoms

Apart from negative family history and age of onset, acquired angioedema cannot be distinguished from hereditary angioedema. People with AAE typically report no family history, and onset is most commonly after the fourth decade of life.

People with AAE have recurrent episodes of edema (swelling) at various locations of the face and/or body, usually lasting 48-72 hours, but they can persist for up to 5 days on rare occasions. The edema usually does not repsond to antihistamines. The frequency of attacks is unpredictable and varies widely among affected people.

Edema may affect subcutaneous tissues (such as the face, hands, arms, legs, genitals, or buttocks). Abdominal organs (such as the stomach, intestines, or bladder) may also be affected and may result in nausea, vomiting, and/or colicky pain and mimic a surgical emergency. The upper airway (such as the larynx) can also be affected. Swelling of the face and extremities is reported most commonly.

When AAE is associated with an underlying disorder such as a lymphoproliferative cancer or connective tissue disease, symptoms associated with those conditions may also be present.

Causes

AAE-I is most frequently associated with B-cell lymphoproliferative disease. To date, only 2 reports of a T-cell lymphoma associated with AAE-I have been documented.

Prognosis

The prognosis of this disease is variable and in some patients with appropriate treatment can be free of disease symptoms for a short time and in some cases patients will be free of symptoms but they have biochemical abnormalities.

Unfortunately there are no published studies that provide information about the long-term outlook (prognosis) for people with AAE. The prognosis can vary and in most cases, it depends on the underlying condition.[2] Compared with the general population, patients with AAE have a higher incidence of B-cell malignancies (cancers). Asphyxiation due to laryngeal edema and upper airway closure is a serious and immediate risk for affected people; while this can cause death, mortality is more likely to be associated with complications of an associated condition.

Treatment

Different drugs are available for the treatment of acquired angioedema. It also needs the intensive support which includes the intravenous fluids and in cases of laryngeal intubation is necessary.