EoE - Apfed

Eosinophilic esophagitis (EoE) is a chronic, allergic inflammatory disease of the esophagus (the tube connecting the mouth to the stomach). It occurs when a type of white blood cell, the eosinophil, accumulates in the esophagus. The elevated number of eosinophils cause injury and inflammation to the esophagus. This damage may make eating difficult or uncomfortable, potentially resulting in poor growth, chronic pain, and/or difficulty swallowing.

What are the symptoms?
Symptoms of EoE may vary from one individual to the next and may differ depending on age. Infants and toddlers often refuse their food or have trouble growing properly. School-age children may have recurring abdominal pain, trouble swallowing, or vomiting. Adolescents and adults most often have difficult or painful swallowing. Their esophagus may narrow and cause food to become stuck (impaction), causing a medical emergency.

Symptoms also may vary given the developmental ability and communication skills of the age group affected.

Common symptoms include:
Reflux that does not respond to medication (acid suppressors) – infant, child, adult
Difficulty swallowing – child, adult
Food impactions (food gets stuck in the esophagus) – older children, adult
Nausea and Vomiting – infant, child, adult
Failure to thrive (poor growth, malnutrition, or weight loss) and poor appetite – infant, child, rarely adult
Abdominal or chest pain – child, adult
Feeding refusal/intolerance or poor appetite – infant, child
Difficulty sleeping due to chest or abdominal pain, reflux, and/or nausea – infant, child, adult

What causes eosinophilic esophagitis?
While the exact cause of EoE is not yet known, the general belief is that it’s typically caused by an immune response to specific foods. Many patients with EoE have food or environmental allergies. Researchers have identified a number of genes that play a role in EoE. These pathways may provide new direction to diagnose, monitor and treat EoE in the future.

Who is affected?
EoE is a recognized disease that is now increasingly diagnosed in children and adults. Eosinophilic esophagitis is a rare disease, but increasing in prevalence with an estimated 1 out of 2,000 people affected. EoE affects people of all ages and ethnic backgrounds. While both males and females may be affected, a higher incidence is seen in males. People with EoE commonly have other allergic diseases such as rhinitis, asthma, and/or eczema. Certain families may have an inherited tendency to develop EoE.

How is eosinophilic esophagitis diagnosed?
A gastroenterologist must evaluate a patient for the symptoms consistent with eosinophilic esophagitis, taking a careful history. Since EoE can mimic other conditions, more common diseases such as gastroesophageal reflux disease (GERD) must first be ruled out.

A doctor will perform an upper endoscopy. With a traditional endoscopy, the patient is either sedated or put under anesthesia, and a small tube called an endoscope is inserted through the mouth. With sedation-free transnasal endoscopy, the endoscope is inserted through the nose. During an endoscopy, the esophagus, stomach, and the first part of the small intestine are examined for tissue injury, inflammation and thickening of the esophageal wall. Small tissue samples are taken (biopsy). This procedure is typically not uncomfortable and may be done on an outpatient basis.

A pathologist will analyze the tissue samples under a high-powered microscope to see the small cell structures. If eosinophils are present in the sample, the pathologist will count how many are visible. A count of 15 or more eosinophils per high-powered microscopic field is highly suggestive of EoE. A patient may have EoE even if the esophagus looks normal during the endoscopy. The biopsies will help in making an accurate diagnosis.

Endoscopy with the biopsies is the only reliable method of diagnosing EoE at this time, although promising research for less invasive diagnostic and monitoring is currently underway.

How is eosinophilic esophagitis treated?

The two main treatments recommended for EoE are diet management and medication. In some cases, a combination of these therapies may be used.

Repeat endoscopies with biopsies are needed to monitor the eﬀectiveness of the treatment plan.

Diet Management:
Diet therapy involves removal of suspected or known food triggers. Often, this includes remove of common allergy-causing foods such as milk, wheat, eggs, soy, peanuts/tree nuts, fish/shellfish. For some patients, elemental nutrition may be recommended to supplement the diet, or for full nutrition. Elemental diets consist of special amino acid-based formulas that do not elicit an allergic response. If a patient has diﬃculty drinking suﬃcient amounts of formula and/or the doctor believes it is necessary for other reasons, there may be the need for placement of a temporary feeding tube (called nasogastric) or a more long-term alternative (called a G-tube).
Medications may include:
- In the U.S., there are two medications indicated to treat EoE were approved by the U.S. Food and Drug Administration: Dupilumab (Dupixent®, a biologic) is approved to treat EoE in patients age 1 and older, and a budesonide oral suspension (Eohilia™) is a medicine approved to treat EoE in people 11 years and older. Talk to your doctor about treatment options.
- Topical steroids (fluticasone or budesonide) swallowed from an asthma inhaler or mixture may help control inflammation and suppress the eosinophils. Systemic corticosteroids such as Prednisone are not used for chronic management of the disorder, but may be prescribed for acute situations and short periods of time. These medications are prescribed “off-label”, meaning they are not specifically indicated for the treatment of EoE.
- Acid suppressors (proton pump inhibitors, commonly abbreviated as PPIs) may also help relieve reflux symptoms in some patients in combination with dietary therapy or medications.
Dilation:
If the esophagus has become too narrow as the result of EoE, it may cause food to become stuck (known as a food impaction). An esophageal dilation may be recommended to treat a narrowed esophagus. This procedure involves inflating a balloon or inserting a tube into the narrowed section of the esophagus and stretching it out.

What is the prognosis?
Eosinophilic esophagitis is a chronic disease that requires ongoing monitoring and management. EoE does not appear to limit life expectancy and there is currently no strong data suggesting EoE causes cancer of the esophagus. In some patients, EoE is complicated by the development of esophageal narrowing (strictures) which may cause food to lodge in the esophagus (impaction). It can also make eating very difficult and uncomfortable for children and adults. It is not clear how long EoE has to exist before strictures form. However, since the natural history of EoE is only emerging, careful monitoring and long-term follow-up is advised.

The initial diagnosis of EoE can be overwhelming and often affects the entire family. A positive attitude and a focus on non-food activities go a long way in learning to live with EoE. With proper treatment, individuals with EoE can successfully manage the condition.

	Eosinophilic Esophagitis: From Biopsy to Microscope
	Upper Gastrointestinal Tract

Other Resources

Educational Content

EoE Brochure (PDF)
EGID Handout. This flyer is designed to teach elementary school-aged children how to support a classmate who has eosinophilic gastrointestinal disease.
APFED EoE Patient Education Kit (PDF) – A great primer for those with a new diagnosis!

Publications

Videos

A guide to successfully managing Eosinophilic Esophagitis
EoE- How do we diagnose?
What Is EoE? by Cincinnati Center for Eosinophilic Disorders
APFED’s Educational Webinar Series
“Eating Without Pain: Partnership for Effective Management of Eosinophilic Esophagitis(EoE)” is available on demand until 2/4/23. An expert panel discusses the symptoms, diagnosis, and treatment of EoE.