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NORD is very grateful to Adriaan Tan, MD, Hepatogastroenterologist, Canisius Wilhelmina Hospital, The Netherlands, for assistance in the preparation of this report.
Eosinophilic gastroenteritis is a rare digestive disease characterized by the triad of eosinophilic infiltration of segments of the gastrointestinal tract, abnormalities of gastrointestinal function (varying from dyspepsia and obstruction to diarrhea and ascites) and exclusion of other diseases with peripheral eosinophilia.
Eosinophilic gastroenteritis may affect any part of the gastrointestinal tract from the esophagus to the rectum. Symptoms include dysphagia (sometimes presenting as food impaction), heartburn, abdominal pain, nausea, vomiting, diarrhea, weight loss, and bloating (ascites is possible). The eosinophilic infiltration may involve one or more layers of the gastrointestinal wall. The particular symptoms present in each case depend upon the layer and the location of involvement. Most commonly, the stomach wall and the small bowel are involved. Mucosal involvement leads to protein-losing enteropathy and malabsorption. Muscle layer involvement causes abdominal pain, vomiting, dyspeptic symptoms and bowel obstruction. Subserosal involvement predominantly causes ascites with marked eosinophilia. Sometimes eosinophilic pleural effusion is present.
The exact cause of eosinophilic gastroenteritis is unknown. Some cases of this disease may be caused by a hypersensitivity to certain foods or other unknown allergens. Often, a family history of allergy is present.
Eosinophilic gastroenteritis is a rare disease (1/100.000) that affects both males and females, but is slightly more common among men. Peak prevalence is in children and adults (20-50 years). The reported prevalence has increased markedly, especially of eosinophilic esophagitis (2-6/100.000). This is probably due to prior under-diagnosis. People with a history of allergies, eczema, and seasonal asthma are more likely to develop this disease.
Symptoms of the following disorders can be similar to those of eosinophilic gastroenteritis. Comparisons may be useful for a differential diagnosis:
Whipple's disease is an uncommon digestive disorder of microbial origin that affects the lining of the small intestine and results in malabsorption of nutrients. This disorder may also affect other organs of the body. (For more information on this disorder, choose "Whipple" as your search term in the Rare Disease Database.)
Celiac sprue is a chronic intestinal malabsorption disorder caused by an intolerance to gluten, an insoluble component of wheat and other grains. Clinical and/or histologic improvement of symptoms follow withdrawal of dietary gluten-containing grains. (For more information on this disorder, choose "Celiac" as your search term in the Rare Disease Database.)
Mastocytosis is a genetic disorder characterized by abnormal accumulations of a particular type of cell (mast cells) normally found in connective tissue. The liver, spleen, lungs, bone, skin and sometimes the membrane surrounding the brain and spine (meninges) may be affected. (For more information on this disorder, choose "Mastocytosis" as your search term in the Rare Disease Database.)
Tropical sprue, a disorder of unknown cause, is characterized by malabsorption, multiple nutritional deficiencies, and abnormalities in the small bowel mucosa. It appears to be acquired and related to environmental and nutritional conditions and is most prevalent in the Caribbean, South India and Southeast Asia. (For more information on this disorder, choose "Tropical Sprue" as your search term in the Rare Disease Database.)
Hypereosinophilic syndrome is characterized by marked blood eosinophilia. It is related to disordered production of myeloid precursor cells or lymphoid T cells. It has a marked clinical heterogenicity. Hematologic malignancy has to be excluded.
Crohn's disease, also known as ileitis, regional enteritis, or granulomatous colitis, is a form of inflammatory bowel disease characterized by severe, chronic inflammation of the wall of the gastrointestinal tract. (For more information on this disorder, choose "Crohn's" as your search term in the Rare Disease Database.)
A careful history may suggest to the physician that a biopsy is required. The results of the biopsy are usually diagnostic.
The corticosteroid drug prednisone is usually an effective treatment for eosinophilic gastroenteritis. Topical steroids can be helpful in eosinophilic esophagitis. Eliminating foods to which a person is allergic may prove helpful in some cases. Surgery may be necessary in severe cases in which there is an obstruction of the intestines. Other treatment is symptomatic and supportive.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
For information about clinical trials sponsored by private sources, contact:
The National Institute of Allergy and Infectious Diseases (NIAID) of the NIH is conducting a clinical research study involving people with eosinophilic gastroenteritis. The study seeks to investigate omalizumab as a possible treatment for people with this disorder. For information, contact the NIH Patient Recruitment Office listed above.
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Tan A. Eosinophilic Gastroenteritis. In: NORD Guide to Rare Disorders. Philadelphia, PA: Lippincott Williams & Wilkins; 2003:342.
Yamada T, Alpers DH, Owyang C, et al., eds. Textbook of Gastroenterology. 2nd ed. Philadephia, PA : J. B. Lippincott Company; 1995:2464-65.
Lucendo AJ. Eosinophilic diseases of the gastrointestinal tract. Scand J Gastroenterology. 2010;45:1013-21.
Bischoff SC. Food allergy and eosinophilic gastroenteritis and colitis. Current Opinion in Allergy and Clinical Immunology. 2010;10:238-45.
Yan BM. Shaffer EA. Primary eosinophilic disorders of the gastrointestinal tract. Gut. 2009;58:721-32.
Gonsalves N, Kahrilas PJ. Eosinophilic oesophagitis in adults. Neurogastroenterol Motility. 2009;21:1017-26.
Mueller S. Classification of eosinophilic gastrointestinal diseases. Best Practice & Research Clinical Gastroenterology. 2008;22:425-440.
Oh HE, Chetty R. Eosinophilic gastroenteritis: a review. J Gastroenterology. 2008;43:741-50.
Nguyen MN T, Szpakowski J-L. Eosinophilic Gastroenteritis. Emedicine. http://emedicine.medscape.com/article/174100-overview. Updated January 4, 2012. Accessed March 7, 2012.
Report last updated: 2012/03/08 00:00:00 GMT+0