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  3. Episodic angioedema with eosinophilia
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Episodic angioedema with eosinophilia


Title




Other Names:
Gleich syndrome
This disease is grouped under:
Hypereosinophilic syndrome

Summary Summary


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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


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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
Do you have updated information on this disease? We want to hear from you.

Cause Cause


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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


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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


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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


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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/
Do you know of an organization? We want to hear from you.

GARD Answers GARD Answers


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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.

Have a question? Contact a GARD Information Specialist.

References References


  1. O'Hollaren MT. The Hypereosinophilic Syndromes. Medscape Reference. 2006; https://www.medscape.org/viewarticle/520117.
  2. 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/.
  3. 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.
  4. 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.
  5. 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/.
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