National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Acquired angioedema


Other Names:
Angioedema, acquired; Acquired C1 inhibitor deficiency
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]
Last updated: 3/8/2018
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.[1]
Last updated: 3/8/2018

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Abdominal pain
Pain in stomach
Stomach pain
[ more ] 		0002027
Angioedema 0100665
5%-29% of people have these symptoms
Ascites
Accumulation of fluid in the abdomen
0001541
Facial edema
Facial puffiness
Facial swelling
[ more ] 		0000282
Intestinal edema 0005225
Intestinal obstruction
Bowel obstruction
Intestinal blockage
[ more ] 		0005214
Laryngeal edema 0012027
Urticaria
Hives
0001025
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Last updated: 7/1/2020

The resources below provide information about treatment options for this condition. If you have questions about which treatment is right for you, talk to your healthcare professional.

Management Guidelines

  • Orphanet Emergency Guidelines is an article which is expert-authored and peer-reviewed that is intended to guide health care professionals in emergency situations involving this condition.  
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.[1]

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.[1][4]

Compared with the general population, people with AAE have a higher chance to develop B-cell lymphomas.[1]
Last updated: 3/8/2018

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
The differential diagnosis should include intestinal occlusion syndrome, hereditary angioedema and histamine-induced angioedema (of allergenic or nonallergenic origin) generally associated with urticaria.
Visit the Orphanet disease page for more information.

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Acquired angioedema. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Patient Registry

  • A registry supports research by collecting of information about patients that share something in common, such as being diagnosed with Acquired angioedema. The type of data collected can vary from registry to registry and is based on the goals and purpose of that registry. Some registries collect contact information while others collect more detailed medical information. Learn more about registries.

    Registries for Acquired angioedema:
    European Society for Immunodeficiencies (ESID) Registry
     

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Merck Manual for health care professionals provides information on Acquired angioedema.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Acquired angioedema. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

  1. Moon AT. Acquired Angioedema Due to C1 Inhibitor Deficiency. Medscape Reference. June 29, 2017; http://emedicine.medscape.com/article/1048887-overview.
  2. Peter J. Delves. Hereditary and Acquired Angioedema. Merck Manuals. March, 2014; http://www.merckmanuals.com/professional/immunology_allergic_disorders/allergic_autoimmune_and_other_hypersensitivity_disorders/hereditary_and_acquired_angioedema.html. Accessed 8/25/2014.
  3. Laurence Bouillet. Acquired Angioedema. Orphanet. August, 2011; Accessed 8/25/2014.
  4. Cicardi M. Acquired C1 inhibitor deficiency: Management and prognosis. UpToDate. Waltham, MA: UpToDate; February 20, 2018; https://www.uptodate.com/contents/acquired-c1-inhibitor-deficiency-management-and-prognosis.