Acquired angioedema

Title

Other Names:

Angioedema, acquired; Acquired C1 inhibitor deficiency

Summary Summary

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.^[1] Swelling episodes may be triggered by mild trauma (e.g., dental work), viral illness, cold exposure, pregnancy, ingestion of certain foods, or emotional stress.^[2] 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.^[1]^[3] Treatment aims to control episodes and normalize C1-INH levels.^[3] When possible, the underlying disorder should be treated.^[1]

Last updated: 8/25/2014

Symptoms Symptoms

Apart from negative family history and age of onset, acquired angioedema (AAE) 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.^[1]

Last updated: 8/25/2014

The Human Phenotype Ontology (HPO) provides the following list of features that have been reported in people with this condition. Much of the information in the HPO comes from Orphanet, a European rare disease database. If available, the list includes a rough estimate of how common a feature is (its frequency). Frequencies are based on a specific study and may not be representative of all studies. You can use the MedlinePlus Medical Dictionary for definitions of the terms below.

Signs and Symptoms	Approximate number of patients (when available)
Angioedema	Very frequent (present in 80%-99% of cases)
Ascites	Occasional (present in 5%-29% of cases)
Facial edema	Occasional (present in 5%-29% of cases)
Intestinal edema	Occasional (present in 5%-29% of cases)
Intestinal obstruction	Occasional (present in 5%-29% of cases)
Laryngeal edema	Occasional (present in 5%-29% of cases)
Urticaria	Occasional (present in 5%-29% of cases)

Last updated: 10/1/2017

Treatment Treatment

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.

Prognosis Prognosis

Unfortunately there are no published studies that provide information about the long-term outlook (prognosis) for people with AAE.^[4] The prognosis can vary and in most cases, it depends on the underlying condition.^[1] Compared with the general population, patients with AAE have a higher incidence of B-cell malignancies (cancers).^[1] 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.^[1]^[4]

Last updated: 8/25/2014

Do you have updated information on this condition? Let us know.

Research Research

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 studying or have studied 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.

Organizations Organizations

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

US Hereditary Angioedema Association, Inc.
Seven Waterfront Plaza
500 Ala Moana Blvd., Suite 400
Honolulu, HI 96813
Telephone: 866-798-5598
E-mail: info@haea.org
Website: http://www.haea.org

Do you know of an organization? Send us your suggestions.

Learn More Learn More

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.

News & Events News & Events

News

Don’t Miss the NCATS Toolkit for Patient-Focused Therapy Development Event
August 31, 2017
Funding Opportunities Now Available for the Undiagnosed Diseases Network (UDN) Phase II
August 30, 2017
IRDiRC Goals 2017-2027: New Rare Disease Research Goals for the Next Decade
August 10, 2017

GARD Answers GARD Answers

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. Submit a new question

What is acquired angioedema? What does it mean to have this disease? Is it life threatening? See answer

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References References

Ru'aa Al Harithy. Acquired Angioedema. Medscape Reference. July 31, 2014; http://emedicine.medscape.com/article/1048887-overview. Accessed 8/25/2014.
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.
Laurence Bouillet. Acquired Angioedema. Orphanet. August, 2011; Accessed 8/25/2014.
Marco Cicardi. Acquired C1 inhibitor deficiency: Management and prognosis. UpToDate. Waltham, MA: UpToDate; August, 2014; Accessed 8/25/2014.