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What is Autoimmune angioedema

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.[1] Swelling episodes may have various triggers, such as mild trauma (such as dental work), viral illness, cold exposure, pregnancy, certain foods, or emotional stress.[2] The frequency of episodes is unpredictable and can vary widely.[1]

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.[1][3] In some cases, it is hard to distinguish between AAE types 1 and 2.[1]

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.[1] During severe or life-threatening episodes, intensive support may be needed (such as IV fluids or intubation for a blocked airway).[1]

This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).


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