Episodic angioedema with eosinophilia

Title

Other Names:

Gleich syndrome

This disease is grouped under:

Hypereosinophilic syndrome

Summary Summary

Episodic angioedema with eosinophilia is a disorder characterized by episodes of swelling under the skin (angioedema) and an elevated number of the white blood cells known as eosinophils (eosinophilia). During these episodes, symptoms of hives (urticaria), fever, swelling, weight gain and eosinophilia may occur. Symptoms usually appear every 3-4 weeks and resolve on their own within several days. Other cells may be elevated during the episodes, such as neutrophils and lymphocytes.^[1]^[2] Although the syndrome is often considered a subtype of the idiopathic hypereosinophilic syndromes, it does not typically have organ involvement or lead to other health concerns.^[1]^[3] The cause of this condition is unknown. Treatment may not be needed, but can involve the use of steroids.^[2]

Last updated: 4/12/2018

Symptoms Symptoms

People with episodic angioedema associated with eosinophilia (EAE) experience recurring episodes of the following symptoms:^[2]^[3]^[4]

Swelling under the skin (angioedema)
Hives (urticaria)
Fever
Swelling
Weight gain
Reduced production of urine

Laboratory findings during these episodes may include:^[2]^[4]

An elevated number of the white blood cells known as eosinophils (eosinophilia)
Elevated levels of certain antibodies, including IgG and IgM

The episodes usually occur every 3-4 weeks and resolve on their own within several days. There may be an increase in urine production once symptoms resolve.^[3]

Last updated: 4/12/2018

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Cause Cause

The cause of episodic angioedema with eosinophilia (EAE) is not well understood. Experts suspect that certain proteins in the immune system called interleukins may play a role. Specifically, interleukin 5 (IL-5) levels are elevated during episodes. Studies have lead to the belief that these proteins play a role in the inflammatory process involved in EAE.^[4]

Last updated: 4/12/2018

Treatment Treatment

Treatment may not be needed for episodic angioedema with eosinophilia (EAE), since symptoms usually resolve on their own. When treatment is initiated, steroids are usually the first choice. Certain medications used to treat cancer, including Imatinib (Gleevac) have also been successfully used in treating EAE.^[4]

Last updated: 4/12/2018

Prognosis Prognosis

Generally, episodic angioedema with eosinophilia (EAE) is considered to be a benign condition with a self limiting course. Typically, other organs are not involved and over time, episodes may resolve on their own.[12728] In some instances, people with EAE can develop symptoms in other organs, such as cardiac disease.^[5] Symptoms may also progress to other types of non episodic hypereosinophilic syndrome.^[3]

Last updated: 4/12/2018

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

American Partnership For Eosinophilic Disorders
PO Box 29545
Atlanta, GA 30359
Telephone: 713-493-7749
E-mail: mail@apfed.org
Website: http://www.apfed.org
International Eosinophil Society
555 East Wells Street, Suite 1100
Milwaukee, WI 53202
Telephone: 414-276-6445
E-mail: info@eosinophil-society.org/
Website: http://www.eosinophil-society.org/

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GARD Answers GARD Answers

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Have a question? Contact a GARD Information Specialist.

References References

O'Hollaren MT. The Hypereosinophilic Syndromes. Medscape Reference. 2006; https://www.medscape.org/viewarticle/520117.
Khoury P, Herold J, Alpaugh A, Dinerman E, Holland-Thomas N, Stoddard J et al. Episodic angioedema with eosinophilia (Gleich syndrome) is a multilineage cell cycling disorder. Hematologica. March, 2015; 100(3):300–307. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4349267/.
Roufosse F, Klion AD & Weller PF. Hypereosinophilic syndromes: Clinical manifestations, pathophysiology, and diagnosis. UpToDate. August 17, 2017; http://www.uptodate.com/contents/hypereosinophilic-syndromes-clinical-manifestations-pathophysiology-and-diagnosis.
Liu F, Hu W, Liu H, Zhang M, Sang H. Episodic angioedema associated with eosinophilia. Anais Brasileiros de Dermatologia. Jul-Aug, 2017; v.92(4):https://www.ncbi.nlm.nih.gov/pubmed/28954105.
Wright BL, Butterfield JH, Leiferman KM, Gleich GJ. Development of Eosinophilic Endomyocardial Disease in a Patient with Episodic Angioedema and Eosinophilia. The journal of allergy and clinical immunology In practice. 2016; 4(2):336-337. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4955938/.