Autoimmune angioedema

From the Autoimmune Association List Only

Summary

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

(Genetic and Rare Disease Information Center, 2021)

Symptoms

Hives

The welts associated with hives can be:

• Skin-colored, reddish on white skin, or purplish on black and brown skin

• Itchy, ranging from mild to intense

• Round, oval or worm-shaped

• As small as a pea or as large as a dinner plate

Most hives appear quickly and go away within 24 hours. This is known as acute hives. Chronic hives can last for months or years.

Angioedema

Angioedema is a reaction similar to hives that affects deeper layers of the skin. It can appear with hives or alone. Signs and symptoms include:

• Welts that form in minutes to hours

• Swelling, especially around the eyes, cheeks or lips

• Mild pain and warmth in the affected areas

(Autoimmune Association, 2022)

Diagnostic Criteria

Under Investigation

Diagnostic Tests

Under Investigation

Human Leukocyte Antigen (HLA) Associations

Under Investigation

Tissue-Type or Cell-Type Attacked

Under Investigation

Attacking Immune Cell Type or Process

Under Investigation

Treatment Modality

Under Investigation

Managing Specialist(s)

Under Investigation

Associated Infections

Under Investigation

Associated Toxins

Under Investigation

Research Authors

Under Investigation

Research Institutions

Under Investigation

Average Time from Symptom Onset to Diagnosis

Under Investigation

Last Updated

January 06, 2023

References

Acquired Angioedema. National Institutes of Health, National Center for Advancing Translational Science, Genetic and Rare Diseases Information Center. (2021, November 08). Retrieved January 06, 2023, from https://rarediseases.info.nih.gov/diseases/8605/acquired-angioedema

Autoimmune angioedema. Autoimmune Association. (2022, November 09). Retrieved January 06, 2023, from https://autoimmune.org/disease-information/autoimmune-angioedema/