Eosinophilic Esophagitis (EoE)
What is EoE?
Eosinophilic Esophagitis (EoE) is a chronic inflammatory disease of the esophagus (the muscular tube that carries food and liquids from your mouth to the stomach). EoE is characterized by the build up of a type of white blood cell, called eosinophils, in the esophagus. This build up causes damage, inflammation, and/or injury to the inner lining of the esophagus which can lead to a variety of symptoms. [1]
Symptoms of EoE can depend on age and will vary from person to person. [1,2,3] For example:
Symptoms in infants and toddlers will typically include more non-specific symptoms like [2]:
Food rejection [2]
Feeding problems/disorders [4]
Reflux that does not get better with medication [4]
Failure to thrive [4]
While symptoms in older children and adolescents typically include:
Trouble swallowing (aka dysphagia) - especially with solid foods [2,4]
Food getting stuck in the esophagus (aka food impaction) [2]
Poor appetite [4]
Less common:
Symptoms in adults often include:
Diagnosis
To find out if you or your child has EoE, your doctor will start by asking about symptoms and taking a detailed medical history. The detailed medical history is an important step in diagnosing EoE, since EoE symptoms are also common in many other conditions. Although there are several validated tools now available to gauge symptoms in adults and children, symptoms alone do not provide an adequate EoE diagnosis. [1]
Because symptoms alone are not enough to diagnose EoE, it is necessary for your doctor to use a combination of an upper endoscopy and biopsy for diagnosis. An endoscopy is a long, flexible tube that has a light and camera at the end of it which will run down your esophagus. A biopsy is done by your doctor during the endoscopy and involves the removal of a small piece of tissue from your esophagus to check for a high number of eosinophils. This is the most reliable way to diagnose the condition because EoE can be present even if the endoscopy appears normal. [1,3]
Prevalence & Causes
EoE has become increasingly common in recent decades. Before 2007, an estimated 15 out of 100,000 individuals were affected by EoE. Since 2017, that number has increased to approximately 63 out of 100,000. The most recent data shows us that there are approximately 5-10 new cases being diagnosed per 100,000 children and adults each year. [2]
While researchers are not certain about the exact cause of EoE, it is widely believed that food and/or environmental allergies play a key role in the development of the condition, with allergic reactions to foods being the main cause of EoE in most people. [2,3,5]
It is important to know that EoE is categorized as a type of food allergy called non-IgE-mediated [6]. Non-IgE mediated allergic reactions are different from IgE mediated allergic reactions, which can cause hives, redness, vomiting, and in severe cases, anaphylaxis, and usually occur within minutes of eating the food. Instead, non-IgE mediated reactions do not appear right away, usually showing symptoms between 2-72 hours after eating the food, and often involve gastrointestinal (GI) tract related symptoms. Read more about food reactions.
Several other risk factors have been reported as possible triggers in developing EoE, including [2]:
Food allergies
Pollen
Early life exposures, infections, alterations in the gut microbiota
Immunotherapy
Proton pump inhibitor (PPI) treatment
Low or borderline levels of vitamin D
Celiac disease
Connective tissue disorders
Autoimmune conditions
Loeys-Dietz syndrome
Genetic factors
EoE & Autism
EoE is part of a rare group of diseases called Eosinophilic gastrointestinal disorders (EGIDs), in which there is an abnormal number of eosinophils affecting the GI tract, from esophagus to colon. [7]
Neurodevelopmental disorders, including autism, are more frequently linked with EGIDs, particularly in childhood and among males. One study found that 30% of patients with EoE also have a neurodevelopmental disorder. [7,8]
Food selectivity and feeding disorders often occur in children with both ASD and EoE. Because selective eating is considered “common” in kids with ASD and often thought to be due to food refusal, food neophobia, and/or texture-based selectivity, EoE may often be overlooked as a possible cause. Some researchers suggest that unidentified EoE may contribute to a child’s selective eating. It’s important to evaluate and rule out any medical causes of selective eating in children with ASD, including EoE.
On the other hand, some researchers suggest that the higher rates of EoE found in kids with autism may be related to them being more likely to have an endoscopy performed, due to increased access to healthcare and diagnostic procedures.[4,9]
Kids on the autism spectrum could also be at higher risk for developing EoE due to inflammatory gut disorders, leaky gut syndrome, and immune dysfunction being more prevalent in this population. [4,9]
For many children with ASD, feeding disorders may be due to a combination of physical, medical and/or behavioral factors and in part caused by long standing pain or discomfort when swallowing or eating. Relieving this discomfort by identifying and treating a potential EoE diagnosis may improve the effectiveness of treatment efforts that are focused on the behavioral aspects. [4]
BOTTOM LINE:
While there is no cure for EoE, identifying and removing triggering foods can be a great way to manage this condition. Children with ASD are more likely to have EoE, and it could impact their food selectivity or development of a feeding disorder. Because selective eating is considered “common” in kids with ASD, EoE may often be overlooked as a possible cause. It is important to evaluate and rule out any medical causes of selective eating, like EoE, in children with ASD.
Next Steps
While there is no cure for EoE, if your child has been diagnosed with EoE, a combination of medication and dietary changes can be used to manage symptoms and prevent further damage to the esophagus. [3,10]
Diet
Because of the role that food allergens play in the development of EoE, dietary changes are considered the primary treatment and are often just as effective as medication. [11,12]
Currently, there are no set guidelines for dietary therapy for EoE. The three common approaches that your practitioner may recommended, depending on your child’s specific needs, are [12]:
1) Empiric Elimination Diet
Involves three phases:
Elimination Phase: Certain foods are removed to help the condition improve, usually for 6-8 weeks, before an endoscopy is done to check the results of the diet. [5,10]
Reintroduction Phase: Food groups are gradually added back in to identify which ones trigger EoE symptoms. [5,10]
Maintenance Phase: Involves long term avoidance of the identified food triggers. [5,10]
These diets are effective for treating EoE and are often preferred by healthcare practitioners, but they can be hard to follow without guidance from a registered dietitian or other professional. [5,10,13]
There are three types of empiric elimination diets that can be followed, with each having its own advantages and challenges. [14]
6-food elimination diet (6FED): Removes the most common food allergens including dairy, wheat or gluten, eggs, legumes, nuts, and seafood.
4-food elimination diet (4FED): Removes dairy, wheat or gluten, eggs, and legumes.
1-food elimination diet (1FED): Removes one common food allergen. This is often cow’s milk, since it has been identified as a major trigger for EoE.
2) Elimination Diet Based on Allergy Test Results
This approach is less common and involves taking an allergy test (skin-prick, serum-specific IgE testing, or atopy patch testing) and removing the foods that the test shows you are allergic to. [13,14]
Research shows that skin-prick tests, serum-specific IgE tests, and patch tests don’t reliably reflect disease activity or identify food triggers for EoE. Since EoE is a non-IgE mediated condition, these tests aren’t always recommended to guide dietary therapy. [5,13] Instead, for people with EoE, IgE allergy tests are mainly used to identify those at risk for immediate allergic reactions to food. [13]
3) Elemental Diet
Under the guidance of a healthcare professional, all regular foods and beverages are removed and replaced by an elemental amino-acid formula. [15]
This should not be a primary treatment approach but may be used as a temporary solution in severe cases. For example, if all other treatments have failed or with toddlers or young children that have active EoE complicated by a failure to thrive diagnosis. [11]
Unpleasant formula taste
High financial cost & lack of insurance coverage
An extensive food reintroduction period requiring several endoscopies
A feeding tube may be required in young children since they are unable to drink enough of the formula to meet their nutrition needs
It can be modified by adding one or two less allergenic foods, like fruits or vegetables, to make it easier to follow. [11]
Additionally, studies show that in some cases a gluten- or casein-free diet can reduce eosinophilic inflammation in the GI tract of autistic children with known food sensitivities. [7]
Keep in mind that restrictive diets can lower a child’s quality of life and be expensive, as alternatives may only be available in specialty stores. Also, multiple follow-up endoscopies are needed during treatment, adding to the cost. [10,11,12]
Supplements
Calcium and vitamin D supplements may be needed if your child is following an elimination diet for EoE and has removed cow’s milk without using a substitute “milk” beverage. [16]
When nuts and/or fish are removed from the diet, consider supplementing with omega-3 fatty acids.
If following an elemental diet, fiber supplements (free of known allergens) can be used for children that develop or are more prone to constipation, since the elemental formulas do not contain fiber. [11]
Medical & Labs
Multidisciplinary Team: EoE is a complex disorder and multidisciplinary management is necessary to get the best level of care you can. Your child’s care team may include pediatricians, gastroenterologists, allergists, pathologists, and registered dietitians that can guide you on lab work, medical procedures, and diet. [5,6]
Medications: Common medications that may be prescribed by your medical provider to treat EoE include: acid suppressors (e.g., proton pump inhibitors), topical corticosteroids, and monoclonal antibodies (for adults and children over age 12). [3,10]
Lab Testing: If failure to thrive or nutritional deficiencies are suspected, biochemical tests (for example, bone and iron metabolism, serum albumin, and prealbumin) may be recommended. [11]
Vitamin D Levels: Some studies have pointed to a higher risk and/or rate of Vitamin D deficiency in people with EoE. [11] This may be related to the known relationship between Vitamin D deficiency and immune-mediated disease, although the link between Vitamin D levels and EoE specifically is unclear and more research is needed. [17,18,19]
Children and adolescents with EoE may benefit from [20]:
Regular monitoring of Vitamin D levels through blood work.
Being educated on sufficient sun exposure to trigger the correct production of Vitamin D.
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Authors
Written by: Cassie Callard, MS, Dietetic Intern
Edited & Reviewed by: Brittyn Coleman, MS, RDN/LD
