Eosinophilic (e-o-sin-o-FILL-ik) esophagitis (EoE) is a recognized chronic allergic/immune condition of the esophagus. The esophagus is the tube that sends food from the mouth to the stomach. In EoE, large numbers of white blood cells called eosinophils are found in the inner lining of the esophagus. Eosinophils can release substances into surrounding tissues that cause inflammation. Normally there are no eosinophils in the esophagus. A person with EoE will have inflammation and increased numbers of eosinophils in the esophagus. The chronic inflammation of EoE leads to symptoms.
The symptoms of EoE vary with age. Infants and toddlers may refuse to eat or not grow properly. School-age children often have decreased appetite, recurring abdominal pain, and trouble swallowing or vomiting. Teenagers and adults can have the same symptoms, but often have difficulty swallowing dry or dense, solid foods. The difficulty swallowing occurs because the esophagus is inflamed, and in severe cases, because the esophagus narrows to the point that food gets stuck. Food firmly stuck in the esophagus is called a “food impaction,” which can be a medical emergency if the food does not pass into the stomach or is not vomited up relatively quickly.
Allergists and gastroenterologists are seeing many more patients with EoE. This is due to an increased incidence of EoE and greater physician awareness. EoE is considered to be a chronic condition that can be treated but is not outgrown.
Eosinophils can be found in the esophageal tissue in diseases other than EoE. One common example is acid reflux disease. Other diseases with eosinophils in the esophagus must be ruled out before EoE can be accurately diagnosed.
Diagnosing Eosinophilic Esophagitis
Currently, performing an upper endoscopy with biopsies of the esophagus is necessary to diagnose EoE. An endoscopy is a medical procedure where a flexible tube containing a light source and a camera lens is passed down the esophagus so your doctor can see if your esophagus is inflamed. Small pieces of esophageal tissue the size of the tip of a pen (biopsies) are taken to be examined under the microscope for the presence of eosinophils and for other signs of inflammation.
There are certain criteria for diagnosing EoE that are followed by allergists, gastroenterologists and pathologists. These include symptoms consistent with EoE in combination with findings on the upper endoscopy and upon examination of the esophageal tissue biopsies by a pathologist that confirm EoE.
Eosinophilic Esophagitis and Allergies
The majority of patients with EoE are atopic. An atopic person is someone who has symptoms of one or more allergic disorders. These include asthma, allergic rhinitis, atopic dermatitis (eczema) and food allergy. EoE may occur in other family members. Because many patients with EoE are atopic, they may be seen first by an allergist who suspects the diagnosis and refers them to a gastroenterologist for confirmation of EoE. Alternatively, if the diagnosis of EoE is made by a gastroenterologist, you may be referred to an allergist for evaluation and treatment of EoE and other accompanying allergic diseases. Seeing an allergist will provide you, your family and the gastroenterologist with information so that any allergic aspects of EoE can be properly treated. An allergist may also help plan diet therapy and eventual reintroduction of foods to your diet.
Eosinophilic Esophagitis: Environmental Allergies
Environmental allergies to substances such as pollens, animals, dust mites and molds possibly play a role in EoE. For some patients, it may seem like their EoE is worse during pollen seasons. Allergy testing for these common environmental allergies is often part of the EoE evaluation.
Eosinophilic Esophagitis: Food Allergies
Immune reactions to foods are the main cause of EoE in most patients. Allergists are experts in evaluating and treating EoE related to food allergies. However, the relationship between food allergy and EoE is complex. In classical Immunoglobulin E (IgE)-mediated food allergy, the triggers are easily diagnosed by a history of a severe allergic reaction such as hives and vomiting within minutes after ingestion of the offending food. In EoE, it is more difficult to establish the role of foods because the reactions are delayed, and can develop over days, making it harder to pinpoint a specific food as the trigger. Foods such as dairy products, wheat, egg, and soy are recognized as the most common triggers for EoE. Unfortunately, skin testing, blood allergy testing, and patch testing do not reliably identify food triggers of EoE. This is because most food allergy reactions in EoE are delayed and caused primarily by immune mechanisms other than classical IgE-mediated food allergy. A person with EoE may have one or more foods triggering their EoE. Once the causative food(s) is (are) identified and removed from a person’s diet, esophageal inflammation and symptoms generally improve in a few weeks. Symptoms may not always reflect whether inflammation is present in the esophagus so additional biopsies are necessary to see if a diet is working and prove which food(s) is (are) causing EoE.
Eosinophilic Esophagitis: Treatment
Empiric Elimination Diets
Eliminating the major food allergens from the diet is considered an acceptable treatment of EoE. The foods excluded usually include foods such as dairy, wheat, egg, and soy. These diets have been shown to be very helpful in treating EoE, although they can be very difficult to follow, especially without the help of a dietitian with experience in dealing with EoE. Foods are typically added back one at a time with follow up endoscopies to help determine which specific foods are causing EoE. Alternatively, diets may start with removing dairy products alone followed by additional foods if inflammation does not resolve.
In this strict elimination diet, all sources of allergens are removed from the diet. The patient receives their nutrition from an amino acid formula alone or sometimes while allowing one to two simple foods chosen based on their low likelihood to trigger EoE. All other foods are removed from the diet. A feeding tube may be needed in very young children treated with this diet, since they are often unable to drink enough of the formula to get adequate nutrition. This approach is generally reserved for children with multiple EoE food triggers who have not responded to other forms of treatment.
Dupixent (dupilumab) is the only medical therapy to date that has been approved by the Food and Drug Administration (FDA) to treat eosinophilic esophagitis. It has recently been approved in patients 12 years of age and older weighing at least 40 kilograms (or about 88 pounds). Dupixent is an injectable medication called a monoclonal antibody (or biologic) that is administered weekly. It reduces the inflammation associated with the disease and improves patients’ ability to swallow food.
Other medications have been shown to reduce the number of eosinophils in the esophagus and improve symptoms. Proton pump inhibitors (PPIs), which reduce acid production in the stomach, have also been found to be able to reduce esophageal inflammation in some patients with EoE. Thus, PPIs are often used as a first treatment for EoE. However, not all patients respond to PPIs, and other forms of therapy such as swallowed topical corticosteroids or food elimination diets are considered. Careful monitoring by a physician knowledgeable in treating EoE is very important.
Corticosteroids, which control inflammation, are helpful medications for treating EoE. Swallowing small doses of corticosteroids so that they come into contact with the inner lining of the esophagus is the most common treatment. Different forms of swallowed corticosteroids are available. Once esophageal inflammation is adequately controlled, the dose of swallowed corticosteroid is decreased to the smallest dose necessary to maintain control of EoE in order to reduce the risk of steroid side effects.
Working with Your Doctors
EoE is a complex disorder. It’s important for patients to listen to their gastroenterologist for advice on managing EoE and figuring out when endoscopies are needed to check to see if the condition is getting better or worse. Patients also need to work closely with their allergist/immunologist to find out if allergies are playing a role. An allergist/immunologist will also be able to tell if you need to avoid any foods and can help you manage related problems like asthma, eczema and allergic rhinitis. It is important that your gastroenterologist and allergist work together and agree on how to take care of your EoE. If you are following a diet to treat your EoE, working with a dietitian who knows about elimination diets used to treat EoE is highly recommended.
When you first find out you have EoE, it can be overwhelming. Families often benefit from participating in support groups and organizations. Visit the American Partnership for Eosinophilic Disorders (APFED) and Campaign Urging Research for Eosinophilic Disease (CURED). These are two lay organizations that provide valuable, reliable resources and have ongoing relationships with the AAAAI.
Your allergist / immunologist can give you more information on EoE, allergy testing and treatment.
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