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Acquired angioedema (AAE) is a rare disorder that causes recurrent episodes of swelling (edema) of the face or body, lasting several days. People with AAE may have swelling of the face, lips, tongue, limbs, or genitals. People with AAE can have edema of the lining of the digestive tract, which can cause abdominal pain and nausea, as well as edema of the upper airway, which can be life-threatening. Swelling episodes may have various triggers, such as mild trauma (such as dental work), viral illness, cold exposure, pregnancy, certain foods, or emotional stress. The frequency of episodes is unpredictable and can vary widely.
There are two forms of AAE. Type 1 is associated with various other diseases including lymphoproliferative disorders, and autoimmune diseases that may not become apparent until years after the angioedema begins. Type 2 is associated with an autoimmune abnormality in which a person has autoantibodies against a protein in the blood called C1-INH. In some cases, it is hard to distinguish between AAE types 1 and 2.
Treatment options depend on the severity of symptoms, the parts of the body affected, and the type of AAE a person has. Various medications may be used to relieve symptoms or prevent complications. When an underlying disease is present, episodes may stop if the underlying disease is treated, but some people continue to experience episodes despite treatment. During severe or life-threatening episodes, intensive support may be needed (such as IV fluids or intubation for a blocked airway).
Source: GARD Last updated on 05-01-20
Episodes of swelling (edema) in people with AAE often begin after the fourth decade of life. Edema can develop in various parts of the face and body. It usually lasts for 2 to 3 days, but rarely may last for up to 5 days. The frequency of episodes is unpredictable and can vary widely among people with AAE, as well as over time in one person.
Edema may develop in the face, lips, tongue, hands, arms, legs, genitals, or buttocks. The face and limbs are most commonly affected. It may also develop in the abdominal organs (such as the stomach, intestines, or bladder), which can cause abdominal pain, nausea, and vomiting. The upper airway can also be affected, which can be life-threatening if the airway becomes blocked.
When AAE is associated with an underlying disease such as a lymphoproliferative disorder or autoimmune disease, other symptoms associated with the underlying disease may also be present.
Last updated on 05-01-20
The long-term outlook (prognosis) can vary, and in most cases, it depends on whether an underlying disease is present. People who are treated for an underlying disease may see improvement or disappearance of symptoms, but sometimes this is only temporary.
The most severe complication of AAE is being deprived of oxygen (asphyxiation) due to significant edema of the upper airway, if this occurs. This can be life-threatening. People with AAE should understand what steps to take in order to get emergency treatment in the case of airway involvement. Because AAE is a rare disorder, few emergency department providers are familiar with its treatment. It may be helpful to remain equipped with a document that briefly explains the diagnosis and outlines the treatment for acute attacks.
Compared with the general population, people with AAE have a higher chance to develop B-cell lymphomas.
Last updated on 05-01-20
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