Eosinophilic esophagitis also known as (EoE) is a chronic disease that occurs in both children and adults resulting in inflammation or irritation of the esophagus (the food tube). It is thought to be primarily caused by the body’s response to certain substances, such as food and environmental (airborne) allergens. Allergens are substances that a person is allergic to such as specific foods, plants, trees etc.
Symptoms of EoE are non-specific and are usually not enough to make a definite diagnosis without additional testing. Infants and toddlers with EoE often have feeding problems, vomiting and poor weight gain. Older school-aged children may have vomiting and abdominal pain, and adolescents may complain of the feeling of food getting stuck (known as dysphagia), especially with solid meals. Dysphagia can lead to food impaction, where food suddenly gets stuck in the esophagus leading to chest pain, choking, drooling and difficulty swallowing. If a food impaction occurs, an emergency endoscopy or scope procedure may be required to remove the food that is stuck.
The cause of EoE is unknown but it is thought to be related to an immune system reaction to specific allergens. Both food allergens and allergens in the air (known as aeroallergens) have been implicated as possible contributing factors.
EoE is more common in boys. Although it can occur in all ethnicities, it has been noted more commonly in Caucasian individuals. It is also seen more commonly in patients who have hay fever or allergic rhinitis, eczema, asthma and food and environmental allergies. EoE may also run in the family.
Taking a thorough medical history is usually enough to raise the suspicion of EoE. However, a definitive diagnosis can only be made by performing a gastrointestinal endoscopy also know as an EGD (esophagogastroduodenoscopy) and taking biopsies in the mid/upper and lower esophagus. Findings seen at the time of endoscopy may include white plaques, furrows, strictures and rings in the esophagus, but these signs are not necessarily specific to the diagnosis of EoE. A biopsy is required to make the diagnosis.
The treatment of EoE consists of:
- Elimination of the triggers of inflammation and
- Treatment or prevention of the underlying inflammation.
Allergy testing is frequently done for patients to identify potential allergens and other allergic diseases because children with EoE also are more likely to have allergies. Allergy testing may include some combination of blood, skin prick and patch tests to screen for potential food and environmental allergens.
Children with EoE may respond to a change in their diet to reduce their exposure to specific items identified as causing problems on allergy testing. Three types of diets may be helpful.
- Elemental diet: strict use of an amino acid/hypoallergenic formula as the sole source of nutrition. These formulas contain proteins that are broken down to their basic building blocks (either peptides or free amino acids) thereby reducing the body’s reaction to them.
- Elimination diet: changing the diet by removing foods that patients may react to, as determined by allergy testing (blood, skin prick or patch testing)
- Common allergen elimination diet: Removing the most common food allergens (soy, milk, wheat, egg, peanut and seafood).
In addition to evaluation by a pediatric allergist, consultation with a registered dietician is useful to be sure that a child or teenager still gets the appropriate amount of calories, vitamins and micronutrients in their diet and to help them maintain the diet. During treatment it is important to monitor an individual’s height, weight and growth because the disease and its treatment may slow the growth of children and adolescents.
Medications that may be used either alone or in conjunction with diet changes include:
Steroids: These medications may be used to decrease the inflammation seen in the esophagus and decrease symptoms of difficulty swallowing, vomiting and gastroesophageal reflux. Depending on the amount of inflammation, steroids may be given orally for a short amount of time. Oral or intravenous (through an IV) steroids are useful in emergency situations for individuals with significant swallowing problems or weight loss, and for those who have been hospitalized for poor intake. Because of the potential side effects of frequent or long-term use, such treatment is not recommended except in more severe cases. Children on long-term steroids should be monitored for potential adverse impact on bone health and growth issues
To minimize the steroid side effects, a topical steroid may be prescribed as a maintenance therapy to keep the inflammation quiet. The medication is typically sprayed from an inhaler and swallowed into the esophagus. A viscous (thick liquid) form of steroids is also available. After using topical steroids, children should rinse out their mouths because residues of the medicine in the mouth can leave them at an increased risk for an oral infection known as thrush, which may worsen feeding difficulties and cause pain. Children on long-term topical steroids should be monitored for growth issues although the effects on growth are minimal or absent due to the very limited amount of topical steroids taken up into the body.
Acid reducing medications: Medications that decrease stomach acid production, which include H2 receptor antagonists or proton pump inhibitors. These may also help with relieving further irritation to the esophagus from stomach acid. They are typically given 1-2 times per day and many different brands are available for over the counter or prescription use. These medicines should be used under the guidance of a physician. Some patients will improve with the use of a proton pump inhibitor.
In some centers, repeat endoscopy may be performed to confirm that the inflammation is gone or is improving. Other centers may choose to follow patients based on their symptom improvement. If endoscopy is being used to determine if a patient is responding, once the endoscopy shows improvement of inflammation, families may be asked to reintroduce one food at a time. This may help to identify specific allergens leading to symptoms and to see if more foods can be tolerated in the diet. Currently, less invasive tests such as blood tests to help follow whether treatments are effective and check for disease recurrence are not readily available.
Newer medications such as humanized monoclonal antibodies to IL-5 (Interleukin- 5) which is given through an intravenous line, are being studied in adult and pediatric patients to see if they are helpful in reducing inflammation and clinical symptoms. These medications are not yet available for clinical use in the United States.
Narrowing of the esophagus due to chronic scarring may occur in some patients with EoE. These are called esophageal stricture(s). These strictures may need to be addressed and treated by endoscopic dilation (stretching) if they do not respond to medicines.
Author(s) and Publication Date(s)
Anthony F. Porto, MD, MPH, Yale-New Haven Children's Hospital, New Haven, CT, and Marsha H. Kay, MD, FACG, Cleveland Clinic, Cleveland, OH – Published July 2013.
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