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Eosinophilic Esophagitis (EoE): Symptoms & Treatment

Eosinophilic Esophagitis (EoE): Symptoms & Treatment

Learn more about the Center for Pediatric Eosinophilic Disorders

What is eosinophilic esophagitis (EoE)?

Eosinophilic (e-o-sin-o-fil-ik) esophagitis (e-so-fa-gi-tis), referred to as EoE, is a chronic allergic inflammatory disease of the esophagus, the muscular tube that carries food from the throat to the stomach. In the past, the condition was referred to as EE.

During an allergic reaction, various cells congregate and cause symptoms like redness, swelling and itchiness. A white blood cell called an eosinophil is one of the types of cells behind an allergic reaction.

What are eosinophils?

Eosinophils are an important part of the body’s immune system. There are always small quantities of eosinophils in the blood and intestine fighting parasites and performing other duties. However, eosinophils can cause problems when they appear in large quantities in areas other than the blood and intestine. 

For example, a child with eosinophils in their nose will have seasonal allergies. If eosinophils are in a child’s lungs, they will have asthma. If the eosinophils are in the esophagus, the child will have EoE.  

An excess of eosinophils can gather in other areas of the gastrointestinal system – such as the large intestine, small intestine and stomach – and cause related, yet distinct, eosinophilic disorders.  

How common is EoE? 

EoE affects children of all ethnicities and family income levels. Children with EoE often have other allergic disorders like asthma, seasonal allergies, food allergies or eczema. 

Thirty years ago, EoE was relatively unknown. Today, EoE is one of the most commonly diagnosed chronic inflammatory diseases of the esophagus and gastrointestinal system. We don’t know whether this is because the disease is becoming more common, or because it is being recognized more often. A combination of the two factors is likely true.

In recent years, diagnoses for EoE have risen dramatically – as much as fivefold since 2009. EoE is now estimated to affect 1 in every 700 people.

What’s the difference between EoE and other eosinophilic disorders?

There are several different types of eosinophilic gastrointestinal diseases (EGIDs), each causing inflammation on a different part of the gastrointestinal system.  

  • EoE affects the esophagus. 
  • Eosinophilic gastritis (EoG) affects the stomach  
  • Eosinophilic enteritis (EoN) affects the small intestine 
  • Eosinophilic colitis (EoC) affects the large intestine 

A related condition – hypereosinophilic syndrome (HES) – is characterized by severe, elevated and persistent eosinophils that cause damage to organs outside the gastrointestinal tract, like the heart and lungs. 

What are the signs and symptoms of eosinophilic esophagitis?

Children with EoE may experience a variety of symptoms, but the most common are: 

  • Difficulty feeding (infants) or difficult eating (children) 
  • Difficulty swallowing, called dysphagia
  • Feeling like food is stuck in the throat after swallowing, known as impaction 
  • A burning feeling in their chest, similar to acid reflux or heartburn 
  • Vomiting
  • Regurgitation of undigested food 
  • Abdominal pain 
  • Nausea 
  • Poor growth, malnutrition and weight loss, which may be labeled “failure to thrive” 

If EoE goes untreated, your child’s throat – specifically their esophagus – may narrow due to scarring. This damage is called stricture.  

How do EoE symptoms differ by age?

The symptoms of EoE can differ by age. For example, infants with EoE may refuse to breastfeed or take a bottle. They may frequently spit up or arch their back – a sign of pain. Because of this, their growth may be slower than normal.

Older children with EoE may be reluctant to eat or eat very slowly. They may develop seemingly irrational aversions to certain foods – even ones they previously enjoyed – and may often complain of heartburn and food “stuck” in their throats. They may also experience trouble sleeping. 

In some cases, EoE is only diagnosed after a child or teen comes to the hospital with food in the esophagus that they are unable to cough up. This is called food impaction. 

EoE shares many symptoms with gastroesophageal reflux disease (GERD), also commonly known as acid reflux. Doctors may first prescribe an acid blocker medication or proton pump inhibitor (PPI) to reduce acid secretion.   

While these medications are highly effective in treating GERD, they do not help children with EoE, so if symptoms persist, medical professionals will know that reflux is not the cause.

It’s estimated that up to 10 percent of children with reflux have EoE.

What are the causes of eosinophilic esophagitis?

While EoE is one of the most commonly diagnosed chronic inflammatory diseases of the esophagus and gastrointestinal system, we still don’t know much about what causes it or why it often seems to run in families. There are several theories, but none have been investigated thoroughly to prove or negate them at this time. 

What are the risk factors of eosinophilic esophagitis?

As our knowledge about EoE and who it affects has grown, so has our understanding of risk factors that may increase the chance your child has of developing EoE esophagus.  

Children with the following conditions may have an increased risk: 

In addition, having a family history of EoE – such as a parent or sibling with the disorder – also increases a child’s risk of developing the disorder.  

How do healthcare providers test and diagnose eosinophilic esophagitis?

If you suspect your child may have EoE, contact your primary care physician. A referral may be made to a pediatric gastroenterologist or pediatric allergist.   

Diagnosing EoE

At this time, EoE can only be diagnosed with a biopsy performed by a gastroenterologist during a pediatric endoscopy. Your child will be sedated for this test, then an endoscope – a thin, flexible tube with a light and camera on the end — will be inserted into your child’s mouth to visualize the esophagus, stomach and beginning of the small intestine.

Visual signs such as rings or white plaque may appear, but tissue can also look normal. For this reason, we always collect small tissue samples (called biopsies) during the procedure. These samples are then sent to the pathology laboratory to diagnose your child. 

Allergy testing

Standard skin and blood allergy tests are not usually effective in diagnosing EoE, due to delayed reactions after ingesting the food. Instead, food allergy tests may be better employed when introducing or re-introducing foods into a child’s diet. Standard allergy testing is useful for other allergic diseases and IgE-mediated food allergies.

What are the treatments for EoE?

In almost all cases, the underlying cause of eosinophilic esophagitis is a food allergy. A gastroenterologist or allergist may recommend dietary restrictions – or avoiding certain foods – to pinpoint the food that triggers your child’s allergic response.    

However, almost all foods have been shown to cause EoE in some patients. And while some patients may be allergic to a single food, others may be allergic to many foods. 

When allergy tests are unable to figure out what’s causing your child’s EoE symptoms, your child’s doctor may recommend they stop eating and instead receive a strict elemental formula for one to three months to heal the esophagus.  

After this, foods are slowly reintroduced in an attempt to discover the food or foods causing the allergy. Repeat endoscopies with biopsy are often necessary.  

Several medications have been tried – including corticosteroids – to treat EoE symptoms. While these medicines may reduce esophageal inflammation when taken, the disease (and symptoms) resume when medication is stopped.

Other possible treatments for EoE include:  

  • Proton pump inhibitors (commonly called PPIs) to reduce stomach acid 
  • Biologics.  
    • Currently dupilumab is the only FDA-approved biologic therapy for EoE in patients 1 year and older. The monoclonal antibody targets IL-4 and IL-13 receptors to reduce inflammation, improve swallowing and lower eosinophil counts. 
    • Another biologic being studied is tezepelumab, a monoclonal antibody treatment approved by the FDA as an add-on treatment for patients with severe asthma in patients age 12 or older. It is now being studied as a potential treatment for patients with EoE. 

Which foods trigger eosinophilic esophagitis symptoms?

The most common food triggers for EoE include: 

  • Milk or dairy 
  • Eggs 
  • Wheat 
  • Soy 
  • Peanuts  
  • Beef 
  • Chicken 
  • Potato 
  • Corn  

Learn more about how to implement an EoE-safe diet for your child.

What are the potential complications of EoE?

Damage to the esophagus from untreated eosinophilic esophagus can cause complications including: 

  • Esophageal perforation: A tear or hole in the esophageal wall. While this may occur from an acute injury, it can also occur over time due to repeated damage. When the esophageal wall ruptures, contents from the esophagus can flow into the bloodstream – causing life-threatening complications. 
  • Esophageal stricture: An abnormal narrowing of the esophagus. When the esophagus becomes narrow or constricted (stricture), it can make swallowing difficult and prevent food from passing through to the stomach. Stricture can cause difficulty swallowing and reflux and is likely to worsen over time. Most strictures can be successfully treated with esophageal dilatation, a procedure to stretch and enlarge the narrowing of the esophagus.

What is the outlook for patients with eosinophilic esophagitis?

With diet modifications — and possibly some lifestyle adaptations centering around food in general — your child with EoE can thrive and live a full life, even if their disease never completely goes away. However, we don’t yet know the long-term effects of EoE. 

Some potential effects of the disease, such as scarring of the esophagus, are troubling. As the current population of children with EoE enters adulthood, we will be able to answer more questions about the disease’s long-term health effects. 

Based on what we know today, most children do not outgrow eosinophilic esophagitis.  

However, ICD-9 codes for eosinophilic diseases were not approved by the National Counsel for Health statistics (NCHS) until July 2008, making it difficult for researchers to accurately track the progress of patients over time. 

With codes now in place, and through collaborative efforts with other pediatric and adult institutions, the long-term outlook for patients with EoE will continue to be an area of active research. 

When should my child see a gastroenterologist and allergist?

Your child with EoE should follow up annually with a gastroenterologist and allergist – even if their disease is well managed and they have no lingering symptoms. If symptoms resume or worsen, or if your child experiences new issues with food impaction, trouble swallowing or allergic reactions, you should contact your child’s doctor right away.

Your visit to the Center for Pediatric Eosinophilic Disorders

Learn what to expect before, during and after your child’s appointment with the Center for Pediatric Eosinophilic Disorders at Children’s Hospital of Philadelphia. See preparing for your child’s appointment.

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