Eosinophilic Esophagitis (EoE)

What Is Eosinophilic Esophagitis (EoE)?

Eosinophilic [EE-oh-sin-oh-FILL-ick] esophagitis [uh-sof-uh-JIE-tis] − “EoE” for short − is an allergic condition that causes swelling in your esophagus (swallowing tube). The esophagus is in an upper part of the gastrointestinal (GI) tract (gut) and is the tube that moves food from your mouth to your stomach when you swallow. EoE happens when a large number of white blood cells called eosinophils [EE-oh-sin-oh-FILLS] collect and cause damage in the lining of the esophagus. It is often an immune response to food, but in some cases, environmental pollen can also trigger EoE.

White blood cells are an important part of our immune system. Eosinophils are a special type of white blood cell. They help us fight off certain types of infections, such as parasites, hookworms, or bacteria. Eosinophils also are involved in other conditions, such as allergies, asthma, and cancer.

Eosinophils serve many roles. Some roles are specific and some are not. This means there are many different processes in the body that can cause eosinophils to be present and/or elevated. Eosinophilic disease may be present when you or your child has high eosinophil counts in the blood or part of the body for a long period of time without a known cause.

Eosinophils can be present in different areas of the GI tract. But eosinophils in the esophagus are not common. Doctors look at the number of these cells in the lining of the esophagus to diagnose EoE.

What Are the Symptoms of EoE?

Symptoms vary from one person to the next, and they may be different depending on age. Symptoms in young children may include problems with eating, vomiting, stomach pain, and poor weight gain. Older children and adults may experience reflux, chest pain, stomach pain, and trouble swallowing. The symptoms can occur days or even weeks after eating a food allergen. But symptoms may also come and go. This can be confusing.

Common EoE symptoms include:

• Reflux that does not respond to usual medical therapy (this includes proton pump inhibitors, which stop acid production in the stomach)
• Difficulty swallowing (dysphagia)
• Food impactions (food gets stuck in the throat)
• Nausea and vomiting
• Failure to thrive (poor growth or weight loss)
• Stomach or chest pain
• Poor appetite
• Malnutrition
• Difficulty sleeping

Who Is At Risk of Developing EoE?

EoE can affect anyone. Boys and men are at a higher risk. In some families, EoE may be passed down through genetics.

EoE has a strong connection to allergies. It is common for people with EoE to have other allergic diseases, such as asthma, allergic rhinitis, or eczema. A variety of triggers may cause too many eosinophils in the esophagus.

How Is EoE Diagnosed?

EoE cannot be diagnosed by symptoms alone. EoE is diagnosed through a test called an upper GI (gastrointestinal) endoscopy done by a gastroenterologist. This test can also be called an EGD (esophagogastroduodenoscopy). Some people call it a “scope” for short. During this procedure, the doctor will take photos and small tissue samples, or biopsies, of the esophagus (and possibly stomach and small intestine) to look for eosinophils. A pathologist reviews the tissue samples under a microscope. If the biopsies show 15 or more eosinophils, then EoE is a possible diagnosis.

When the doctor performs these tests, the surface of the esophagus may appear very red and swollen. It could be very narrowed (esophageal rings and or strictures). It could have many white bumpy patches. It might even have an accordion-like appearance.

Other diseases can cause eosinophils in the esophagus as well. Some other causes include:

• Gastroesophageal reflux disease (GERD)
• Other forms of esophagitis that damage the lining of the esophagus
• Infection
• Inflammatory bowel disease
  
  

GERD typically causes far fewer eosinophils in the esophagus than EoE. Currently, experts have not determined what range of eosinophils indicates severe reflux or a process that may respond best to anti-acid medication, and what range clearly indicates EoE. Unfortunately, no other cell or tissue findings have been identified to help clarify this situation. Before EoE can be diagnosed, your doctor may try GERD treatment. It is important for other conditions with similar symptoms, such as GERD, to be ruled out before EoE can be accurately diagnosed.

How Is EoE Treated?

If you are diagnosed with EoE, you may have a team of specialists who help you manage your condition, such as a gastroenterologist, allergist, and a registered dietician.

There are two main ways to treat EoE:

• Dietary changes
• Medicines

These treatments may be used together.

Dietary Changes for Treating EoE

EoE is often triggered by food. Many children and adults with EoE respond well to changes in their diet. Foods that cause symptoms are generally removed from the diet, especially those that cause trouble swallowing or can become stuck.

There are three main types of dietary therapy for EoE:

1. Empiric elimination diet – This is a very effective treatment that involves avoiding major food allergens. Your doctor may have you try this diet without allergy testing. There are different versions of this diet. The six-food elimination diet removes milk, egg, wheat, soy, nuts, and seafood. Another version removes four allergens: milk, egg, wheat, and soy. Or your doctor might have you remove only cow’s milk. According to studies, the four-food elimination diet is the most effective dietary therapy for EoE.¹
   
   
2. Testing-directed elimination diet – In some cases, food allergy testing may help determine which foods to avoid. Before starting this diet, your doctor will do allergy testing to identify food allergies. Two main types of allergy tests may be used: skin prick tests (“scratch tests”) and specific IgE (sIgE) blood test. Both tests check for specific allergic responses to foods. If you or your child tests positive to a food, the doctor will usually recommend removing that food from the diet. The doctor may also recommend you remove cow’s milk because it can often show as a false negative on allergy tests.
   
   
3. Elemental diet – An elemental diet is a liquid, formula-based diet. This special formula does not include any food-based sources of protein. An elemental diet includes only an amino acid (elemental) formula, such as Neocate^®, Elecare^®, ALFAMINO^®, or Pure Amino^™. Your doctor may only allow simple sugars, salt, oils, and certain fruits on an elemental diet. Some people require a feeding tube to help with this type of diet. An elemental diet is difficult to follow. It is usually only recommended for people who don’t respond to other therapies.
   
   

All three diets appear to work quite well to get rid of symptoms and eosinophils. Having three choices gives doctors and families many options to explore. If you or your child has EoE, talk with your doctors about these choices. Together, you can find a treatment choice that will work best for you or your child.

After some time on dietary therapy, you may be able to add food back to your diet by doing a food trial. In a food trial, you will add food back one ingredient at a time. This helps determine which foods are causing a reaction. Food trials begin after symptoms go away from diet therapy. If symptoms return, the doctor may perform another biopsy to see if eosinophils have returned. It is important to remember that the foods that trigger EoE can change over time.

If a dietary approach is taken, work with a registered dietician if possible. Elimination and elemental diets can result in an unhealthy restriction of calories and important nutrients. The overall goal is to maintain a healthy, balanced diet. Dietary therapy may be used as the only therapy for EoE, or it can be combined with medicines.

Medicines for Treating EoE

Medicines can also help manage and reduce symptoms associated with EoE. Your doctor may first prescribe a proton pump inhibitor (PPI), such as omeprazole. PPIs can help keep eosinophils from collecting in the esophagus in children as young as 1 year old. They are commonly used for several weeks.

Corticosteroids can help reduce the swelling in the esophagus. Fluticasone and budesonide are an inhaled corticosteroid (ICS) often used for asthma but when they are used for EoE, instead of being inhaled, they are swallowed. When using an ICS like fluticasone for EoE, a spacer or valved holding chamber is not needed or recommended. When using budesonide for EoE, the nebulizer machine is not needed or recommended. The goal is to get the medicine into the esophagus by swallowing and not into the lungs by inhaling. Do not eat or drink for 30 minutes after using an ICS for EoE.

What Happens After an EoE Diagnosis?

Schedule follow-up visits with your doctor to discuss the best plan. Visits should be every one to three months after diagnosis. The number of visits will depend on how severe the disease is. Once on a treatment plan, follow-up visits may be every six months to one year. These visits will focus on answering your questions and how well treatment is working. This includes symptoms and response to medications. The doctor will check your child's weight and overall nutrition. They will also check to see if new problems in your child’s GI tract have developed.

At each visit, discuss how well diet therapy is working. If symptoms have been better for six months to a year, you or your child may be ready for a food trial. Ask if it is time to add restricted food back into the diet, or to change elemental formulas. A gastroenterologist or allergist can work with you to help make these decisions.

Review the medicines you or your child are taking with the allergist. PPIs are used for four to eight weeks. They can be stopped when you or your child feel better but may be needed in the future if symptoms come back. Corticosteroids can also be decreased and stopped when symptoms improve and resolve.

You can also work with your doctor to decide if another endoscopy is needed. If there are problems with swallowing or food impactions, follow-up scopes may be needed for ongoing monitoring and treatment.

An EoE diagnosis can be overwhelming. The burden of food allergies can impact a family’s financial, emotional, and social well-being. EoE can be especially challenging because of strict dietary restrictions combined with balancing your child’s nutrition.

Medical and dietary treatment alone are not enough to help you deal with the stress of managing EoE. It’s important that you find additional support and resources. Kids With Food Allergies (KFA) offers several resources to help you manage your child’s condition:

• KFA community – Join our online support community to talk with other families managing food allergies in an encouraging environment. When you join our community, you’ll also receive notifications about news, research, food recalls, and educational information to help you manage your child’s food allergies.
  
  
• Formula Options for Infants and Toddlers With Food Allergies – Learn more about different types of formula options available, as well as information on what to do if you can’t afford formula.
  
  
• Safe Eats® Allergy-Friendly Recipes – Search our collection of more than 1,500 recipes that can be made free of several of the most common allergens. You can search to meet your child’s dietary needs or browse by category.
  
  
• Allergy-Friendly Foods – Search our collection of allergy-friendly foods from several different allergy-friendly food companies. You can search for foods free of your child’s allergens or by type of food.

Download the “My Life With Food Allergy Parent Survey Report: Spotlight on Eosinophilic Esophagitis”

Medical Review: May 2021 by Michael Pistiner, MD, MMSc

References
1. Hirano I, Chan ES, Rank MA, Sharaf RN, Stollman NH, Stukus DR, Wang K, Greenhawt M, Falck-Ytter YT; AGA Institute Clinical Guidelines Committee; Joint Task Force on Allergy-Immunology Practice Parameters. AGA institute and the joint task force on allergy-immunology practice parameters clinical guidelines for the management of eosinophilic esophagitis. Ann Allergy Asthma Immunol. 2020 May;124(5):416-423. doi: 10.1016/j.anai.2020.03.020. PMID: 32336462.