• Eosinophilic esophagitis (EoE) occurs when white blood cells called eosinophils build up in the esophagus and cause inflammation.
• Symptoms of EoE vary by age but generally include difficulty swallowing, as well as vomiting and reflux.
• Treatment options for managing symptoms include one therapy for EoE approved by the U.S. Food and Drug Administration (FDA).

Eosinophilic esophagitis is an inflammatory disease caused by an excess of immune cells, known as eosinophils, in the esophagus. According to the National Organization of Rare Disorders (NORD), this chronic condition affects around 1 in 2,000 people. People of any age can develop EoE, and having other allergic conditions increases your risk. Fortunately, several treatment options can both target EoE inflammation directly and manage symptoms of the condition.

What Is Eosinophilic Esophagitis?

EoE develops when an overactive immune system affects esophageal tissues. The immune system acts as the body’s first line of defense against invaders such as bacteria and viruses. However, in immune-related diseases such as EoE, the immune system can go into overdrive and begin harming the body’s healthy tissues as well.

In eosinophilic esophagitis, too many eosinophils — a specific type of white blood cell — build up in the esophagus (the tube that connects the mouth to the stomach). Eosinophils normally help fight infections by creating inflammation, trapping invaders, and releasing toxic substances to kill the invaders. However, with EoE, excess eosinophils in the esophagus create inflammation, damaging that part of the digestive system.

While researchers aren’t sure exactly what causes EoE, they believe the eosinophils are related to an allergic reaction to certain substances, such as:

• Pollen
• Mold
• Dander (skin flakes from animals)
• Dust mites
• Common food allergens (substances that trigger an allergic reaction)

Exposure to these allergens while breathing or eating may set off a reaction, leading to EoE symptoms.

What Are the Symptoms of Eosinophilic Esophagitis?

Symptoms of eosinophilic esophagitis vary from person to person, especially across age groups and between pediatric and adult cases. Symptoms arise mainly as a result of inflammation in the esophagus, which leads to strictures or narrowing of the tube. This prevents food from moving down into the stomach, causing complications with eating and digestion.

For infants and young children, symptoms can include regurgitation (vomiting), difficulty feeding, reflux that doesn’t improve with medication, and problems with growth and weight gain.

For older children and adolescents, symptoms include abdominal pain, vomiting, dysphagia (difficulty swallowing), and reflux.

For adults, symptoms include dysphagia, food impaction (getting stuck in the esophagus), and chest pain from heartburn and reflux.

Who Is at Risk of Developing Eosinophilic Esophagitis?

Certain factors increase a person’s risk of developing eosinophilic esophagitis. Genetics play a large role in many diseases, including EoE. You’re more likely to develop the condition if you have a family member with EoE. Researchers have found that having certain genes that are related to eosinophil function can also increase the risk of EoE.

EoE can develop at any age in any person, but white males ages 30 to 40 are more likely to be diagnosed with the condition. Risk is also higher for those who have other immune-related or allergic conditions, including:

• Atopic dermatitis (eczema)
• Food allergies
• Asthma
• Environmental allergies, such as allergic rhinitis (seasonal allergies)

Recognizing the symptoms of EoE can help point your doctor toward a correct diagnosis.

How Is Eosinophilic Esophagitis Diagnosed?

If your doctor suspects that you have EoE, they will likely refer you to a gastroenterologist. This type of doctor specializes in diseases that affect the gastrointestinal system, including the esophagus, stomach, and intestines. Because EoE is an allergic condition as well, you may also be referred to an allergist for treatment.

Eosinophilic esophagitis usually becomes apparent because a person has difficulty swallowing. A gastroenterologist will perform an upper endoscopy to look for narrowing in the esophagus, which may also have multiple rings. A biopsy of the esophagus must be done to confirm the diagnosis, with the pathologist looking for increased eosinophils.

Blood Tests

If your allergist believes that a specific allergy is causing your EoE, they may do blood tests to check. These tests look for the antibody immunoglobulin E (IgE), which the body makes in response to a specific allergen. They may also check to see if you have higher-than-normal eosinophil counts.

Upper Endoscopy

To get a look at the esophagus and any inflammation or strictures, your gastroenterologist will perform an upper endoscopy. This procedure involves a long, thin tube with a camera attached to one end. The tube is inserted into the mouth and down the esophagus to provide a view of the tissues and take pictures.

Esophageal Biopsy

During an upper endoscopy, your gastroenterologist will take a tissue sample from the esophagus. Known as a biopsy, this technique can help determine whether you have EoE or another condition. The tissue sample is sent off to a lab, where another specialist physician called a pathologist looks at it under a microscope to count the number of eosinophils.

What Are the Treatments for Eosinophilic Esophagitis?

After you are given a diagnosis of EoE, your gastroenterologist will discuss your treatment options with you. Currently, just one therapy is approved by the FDA specifically for the treatment of EoE. Other nonspecific treatments also might help alleviate symptoms. Some treatments may work better than others, so it’s important to remember that this can involve some trial and error.

Dietary Therapy

For people whose EoE is caused by food allergies, eliminating certain allergens that trigger a response may help manage EoE. Doctors might have you try a few dietary strategies, including elimination and elemental diets. Although dietary treatments can be effective for EoE, they are restrictive and can make it difficult to maintain proper nutrition.

Proton Pump Inhibitors

Proton pump inhibitors (PPIs) are medications typically used to treat gastroesophageal reflux disease (GERD). They reduce the amount of acid the stomach makes, which can help alleviate EoE symptoms such as heartburn. Doctors often start with PPIs to treat EoE, but for many people, these drugs don’t relieve EoE symptoms.

Topical Steroids

Medications called steroids help control inflammation. In EoE, topical steroids are swallowed to act directly on the esophagus to help dampen inflammation. Budesonide (Entocort EC) is usually taken in a slurry — a liquid with a thickener, such as a sweetener to help it taste better. Fluticasone (Flovent), a powder, requires an inhaler.

Dupilumab

Dupilumab (Dupixent) is a biologic medication approved by the FDA for treating EoE in adults and children ages 12 and older. This human-made antibody works by blocking inflammation, treating the cause of EoE at its source. Dupixent is the first and only FDA-approved EoE treatment. In clinical trials — research studies to evaluate treatments — dupilumab successfully reduced EoE symptoms and helped participants achieve remission compared with a placebo.

Esophageal Dilation

If your esophagus is extremely narrow from strictures or topical steroids don’t work well enough on their own, your doctor may recommend esophageal dilation. This procedure opens the esophagus by stretching it out. While this method can help avoid use of topical steroids, it often needs to be repeated multiple times to be effective.

Find Your Team

On myEoEcenter, the site for people with eosinophilic esophagitis and their loved ones, people come together to learn more about EoE and share their stories with others who understand life with EoE.

Have you or a loved one been diagnosed with EoE? Do you have symptoms that you believe could be caused by EoE? Share your experiences in the comments below.