Eosinophilic esophagitis, or EE for short is a disorder that affects people of all ages and involves a type of allergic reaction to food. EE has been getting an increasing amount of attention over the past 10 years from doctors and nutritionists as well as from the general public. There has been a dramatic rise in the number of new cases in the United States, Canada, Australia and parts of Europe. Current best estimates are that EE occurs in at least 10,000 people.
What is EE?
EE gets its name from eosinophils, which are a type of white blood cell that indicate allergic inflammation. Eosinophils can normally be found in our bloodstream and tissues, but healthy people do not have any in their esophagus. The presence of a few eosinophils in the esophagus often signifies gastroesophagal reflux (heartburn) while the presence of many eosinophils confined to the esophagus is generally a sign of EE.
So, how do you know if you or someone you love has EE? The symptoms vary, depending on the age of the person.
With infants and toddlers, families often note feeding difficulties, irritability, and, occasionally, poor weight gain. Older children typically have regurgitation, vomiting, heartburn and ‘belly pain’.
While teenagers and adults can also show symptoms similar to those of older children, they more typically have chest pain with or without eating, difficulty swallowing, and a feeling that their food gets stuck while swallowing.
In most cases, patients with EE do not fully respond to acid-blocking medications such as H2-blockers. Interestingly more than half also have asthma, IgE-mediated food allergies, eczema, or allergic rhinitis.
Further investigation and initial treatment
Once the diagnosis of EE is suspected the patient should be referred to a gastroenterologist, who will usually order an x-ray study of the upper gastrointestinal tract (commonly referred to as an upper GI series) to provide physical details of the esophagus, stomach and duodenum.
Unless the x-rays reveal a distinct abnormality, the patient is prescribed medication to suppress stomach acid and is monitored by the doctor.
If symptoms persist or the patient can’t be weaned off the medication, an upper endoscopy is performed, which allows the doctor to examine the esophagus through a special scope.
However, since a normal-appearing esophagus does not rule out the presence of eosinophils or the diagnosis of EE, biopsies of the esophagus, stomach, and duodenum are also obtained, so that the doctor can look for the presence of abnormal cells.
EE is diagnosed when the number of eosinophils in the esophagus exceeds an established threshold in those who also show symptoms of this disorder.
Once diagnosed with EE the patient is referred to an allergist to be evaluated for food allergies and other related conditions.
Unlike patients with recurring heartburn (gastroesophagal reflux) who respond to medication, EE patients respond to the removal of foods from their diets.
Unlike patients with typical food allergies. EE patients do not experience severe hives, lip swelling, coughing, wheezing, immediate vomiting, or anaphylaxis, unless they also have a severe allergy to specific foods (such as peanuts).
Delayed allergic response
Patients with EE typically have a delayed allergic response to a food or foods.
The actual mechanisms in the reactions are different - one being immediate (IgE mediated and sometimes anaphylaxic) and the other being delayed, or non-IgE mediated (EE).
In patients with EE, the allergist will evaluate both types of reaction by using skin-prick testing (IgE-mediated) and, in some cases, atopy patch testing (non-IgE mediated) for foods in the diet.
Environmental allergens are also addressed and managed by the allergist. However, it is rare for them to play a role in EE.
Steroid and dietary manipulations
The treatment of EE includes medication and diet modification.
Steroids taken in a pill form (prednisolone) or swallowed as a topical spray (Flovent® and Beclomethasone) have been shown to relieve symptoms and tissue damage in many patients. Unfortunately, when these medications are discontinued, the disease almost always recurs.
Other drugs, such as Cromolyn sodium (Intal®) and montelukast (Singular®) have not been shown to be effective.
Diet modification - both restricted diets and total elimination diets - have proven successful in the majority of patients with EE.
A treatment strategy for EE includes:
1. Use of an acid-blocking
2. Evaluation by an allergist to determine food allergies.
3. Dietary modification (with strict adherence to the diet).
4. Evaluation by a nutritionist.
A follow-up endoscopy with biopsy is recommended six weeks after beginning treatment, to determine if the suspected foods are in fact
causing the disorder.
This type of follow-up is repeated at intervals to keep track of the patient’s progress, because an improvement in symptoms does not always mean the the condition itself has improved.
Unfortunately, there are currently, no non-invasive methods for the physician to use to follow the progress of someone with EE.
People often ask what will happen if the disorder goes untreated. The answer is that we don’t know. Based on what we know to date, there doesn't appear to be an increased risk of increased harm, although if EE is not controlled, difficulty swallowing, narrowing of the esophagus, and strictures may result.
Ten year review
We recently published the results of a ten year study we conducted at the Children’s Hospital of Philadelphia involving 381 children with EE. In that study, we found that 82% of these children experienced gastroesophagal reflux (either vomiting or heartburn) and 18% had difficulty with swallowing or with food ‘getting stuck’.
We reviewed the medications they were given for EE over the years, and found that cromolyn and montelukast were not effective. Steroids were effective, although both the symptoms and the eosinophils returned in most patients when they stopped taking the steroid. Most of our patients went on restricted diets, based on the results of skin and patch tests, and approximately 70% of these patients improved as a result of this treatment.
Others - including those who didn’t respond to dietary restriction - went on a more drastic diet, in which their food intake was confined to an amino-acid-based formula such as Neocate or Elecare and 97% of these patients improved as a result.
In summary, EE is an emerging disorder that affects all ages. Awareness, recognition and management of EE are current topics of discussion in the fields of allergy and gastroenterology. Genetics, underlying abnormalities and new therapies such as anti-IL5 (which inhibits the development and function of eosinophils) are being researched.
In addition, the American Partnership for Eosinophilic Disorders (www.apfed.org) provides families with support, advocacy and factual, timely information on EE. It also helps to support research.
Courtesy of FAAN www.foodallergy.org
For further information on eosinophilic diseases check
First published in 2007
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