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Genetic and Rare Diseases Information Center (GARD)

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Eosinophilic enteropathy

Other Names for this Disease
  • Eosinophilic enteritis
  • Eosinophilic esophagitis
  • Eosinophilic gastritis
  • Eosinophilic gastroenteritis
  • Eosinophilic gastroenteropathy
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Your Question

I have recently been diagnosed with eosinophilic enteritis by doctors after 3 years of symptoms.    My doctors have had limited experience with this rare disease.   I would like to learn more about the disease course and treatment options.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is eosinophilic enteropathy?

Eosinophilic enteropathy is a condition that causes a type of white blood cell called an eosinophil to build up in the gastrointestinal system and in the blood. Eosinophils play a role in the body’s immune response by releasing toxins. Eosinophils are associated with allergic-type reactions, but their specific function is largely unknown.When eosinophils build up in the gastrointestinal tract, this begins to affect the body by causing polyps, tissue break down, inflammation, and ulcers. Eosinophilic enteropathy can occur in children or adults and is characterized by intolerance to some foods. Eosinophilic enteropathy can affect any part of the gastrointestinal tract, and is often named by the part affected: colon (colitis), esophagus (esophagitis), stomach (gastritis), or both the stomach and small intestine (gastroenteritis).[1][2]
Last updated: 2/25/2011

What are the signs and symptoms of eosinophilic enteropathy?

The symptoms of eosinophilic gastroenteritis vary depending on where the eosinophils build up in the gastrointestinal system and which “layers” of the intestinal wall are involved. Symptoms often include pain, skin rash, acid reflux, anemia, diarrhea, stomach cramps, bleeding, nausea, vomiting, loss of appetite, blood loss in stools, and choking. Symptoms can occur at any age, although they usually develop between ages 20 and 50 years. The symptoms of eosinophilic enteropathy overlap with other gastrointestinal disorders, such as ulcerative colitis, which makes diagnosis difficult. It is common for individuals with this disorder to have symptoms for many years before an accurate diagnosis is made.[1][2][3]
Last updated: 2/25/2011

How is eosinophilic enteropathy diagnosed?

Endoscopy and biopsy is the only way to confirm the diagnosis of eosinophilic enteropathy. During an endoscopy, a gastroenterologist looks at the gastrointestinal tract through an endoscope and takes multiple small samples (biopsies), which a pathologist reviews. A high number of eosinophils suggests the diagnosis of eosinophilic enteropathy. The pathologist will also look at the location of the eosinophils, changes in the tissue layers, and degranulation (spilling of the contents of the eosinophils). Eosinophils may be normally found in small numbers in all areas of the gastrointestinal tract except the esophagus. However, the number of eosinophils seen in individuals with eosinophilic enteropathy is much higher. Once the diagnosis of eosinophilic enteropathy is confirmed, food allergy testing is typically recommended to guide treatment. Tests for food allergies include skin prick testing, patch testing, and a Radioallergosorbent test (RAST).[2]
Last updated: 2/25/2011

How might eosinophilic enteropathy be treated?

There is no "cure" for eosinophilic enteropathy, but treatment can help alleviate symptoms and prevent further damage to the gastrointestinal tract. Treatment of eosinophilic enteropathy varies based on the location of the eosinophils, severity of symptoms, and other medical problems the child or adult may have. In most cases, dietary restrictions and medications can significantly improve the problematic symptoms of this condition.[2]

Food allergy testing is used as a guide for restriction or elimination diets. An elimination diet means strictly avoiding all foods to which the patient has tested positive on allergy testing. Skin and patch testing are used to guide elimination diets.[2]

Sometimes a stricter diet, called an elemental diet, is needed. Skin and patch testing are used to guide elimination diets, but it only takes one false negative food for the diet to "fail". Elemental diets are diets that do not include whole or broken-down forms of protein. Instead, special elemental formulas are used, which are made of amino acids (the building blocks of proteins), fats, sugars, vitamins and minerals. Amino acids do not cause allergic reactions but whole or partial proteins can.[2]

Children and adults who rely in part, or completely, on an elemental amino acid based formula may have a difficult time drinking enough of the formula. To maintain proper nutrition, some require tube feedings directly into the stomach (enteral feeds). In the most severe cases, nutrition is administered directly into the blood stream (parenteral feeds).[2]

The American Partnership for Eosinophilic Disorders provides more information about treatment for eosinophilic enteropathy. This organization also provides more details on restricted or elimination diets and elemental diets.
Last updated: 6/3/2011

What is the disease course for eosinophilic enteropathy?

Little is known about the disease course of eosinophilic enteropathy as individuals with this condition have not yet been evaluated over a long period of time. However, from currently available information, it is likely that some patients completely recover from this condition, and others experience persistent disease. When these diseases present in infancy and specific food sensitivities can be identified, the outlook appears favorable with possible remission in later childhood. It is possible for different parts of the gastrointestinal tract to be affected over time so regular endoscopic and cardiopulmonary (heart and lung) evaluations are recommended.[1]
Last updated: 2/25/2011