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What
is Eosinophilic Esophagitis (EoE)?
(pronounced “ee-oh-sin-oh-fill-ick
ee-sof-uh-ji-tis”)
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Eosinophilic Esophagitis (“EoE”,
formerly “EE”), also called Eosinophilic Oesophagitis in the UK, is an
allergic inflammatory disease characterized by an infiltration of increased
numbers of eosinophils in the esophagus (the tube that runs from the
mouth to the stomach). This
infiltration causes inflammation of the tissue lining the esophagus. In a healthy person, there are
absolutely no eosinophils in the esophagus. There can be a few in the lining
of the gut, but there should be none in the esophagus. A few can be found
with reflux. A biopsy with an eosinophil
count of 15-20 or more per high power field (under the microscope), may be
caused by Eosinophilic Esophagitis.
Other potential causes are Gastroesophageal Reflux Disease (GERD),
food allergies and irritable bowel disease.
Unfortunately, many people go undiagnosed
for years, suffering with what can be severe symptoms. Some people aren’t
diagnosed until they are teens or adults and seek treatment when food has
become impacted in their esophagus. Studies at Cincinnati Children’s Hospital have shown that Eosinophilic Esophagitis is even more common than other well known diseases that affect the gastrointestinal tract such as Crohn's Disease and Cystic Fibrosis. Eosinophilic Esophagitis is the most prevalent of the Eosinophilic Gastrointestinal Disorders. There are at least two types of Eosinophilic Esophagitis: Allergic (responds to food elimination and returns when the trigger foods are returned to the diet) and non-allergic (no response to food elimination). Researchers now are finding that there may be a third type, which is specifically caused by reflux and responds to proton pump inhibitors (PPIs). The current (*) estimated prevalence of Eosinophilic
Esophagitis is 1 in 2000. (*estimate as of 2007) |
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Symptoms of Eosinophilic Esophagitis include:
Inhibitors, such as Prevacid,
Prilosec and Protonix)
In addition to the
above, many people also experience pain in their lower limbs (legs, ankles
& feet) which is not uncommon with GI disorders in general, and fevers (likely
caused by internal inflammation). Some
have also experienced ear infections, asthma, croup, migraines, and more
frequent “colds” when they are reacting to a food. Behavioral changes
have also been reported in some children (*). Associated conditions
can include eczema, rhinitis, regular food allergies (causing the more common
immediate reactions), asthma / reactive airway problems, failure to thrive
and developmental delays (*). (*If
your child has ongoing behavioral issues, social skills difficulties or any
developmental issues, you should speak to your child’s Drs. and consider an
evaluation by a Developmental Pediatrician.) |
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Diagnosis: A diagnosis is made when an
endoscopy is performed (by a Gastroenterologist) and multiple biopsies are
taken. Eosinophilic Esophagitis can
NOT be diagnosed by symptoms alone. An
endoscopy with biopsies is the only way to properly diagnose EoE. Even if the esophagus looks fine to
the person performing the scope, the biopsies may still show eosinophilic
infiltration, indicative of Eosinophilic Esophagitis (EoE). It should be noted that even if the
esophagus looks normal, the presence of eosinophils (which would be seen in
the biopsies) can, and often will, still cause symptoms. However, the Dr. may also see rings or
furrowing, thickened folds, microabcesses, white plaques, etc. The pathologist will also look for tissue
injury, swelling and thickening of the esophageal layers. With Eosinophilic
Esophagitis, the eosinophils are limited to the esophagus and not found in
other areas. The formal diagnostic criteria
should be in place soon, but Eosinophilic Esophagitis can be diagnosed when
the number of eosinophils in a esophageal biopsy is greater than 15 – 20 per
HPF (high power field) under the microscope.
Multiple biopsies need to be taken, as it tends to present in
patches. It is recommended that 4-5
biopsies be taken from each area of the esophagus: 4-5 from the top (proximal), 4-5 from the
middle, and 4-5 from the bottom (distal esophagus), because with any less, it
may be missed. Once Eosinophilic Esophagitis has
been diagnosed, food allergy testing is usually recommended to guide
treatment. Skin prick testing (SPT) to different foods is the most common
type of allergy testing, and may prove helpful, but EoE is caused by a
delayed reaction, not an immediate reaction as is tested for via SPT. Patch testing, which looks for delayed
reactions, is also being used with some success. However, it is not uncommon to have
negative allergy testing and still react to those foods that are being
tested. An EoE reaction can range from
days to weeks as the number of eosinophils increase and cause damage to the
esophagus. Please note that if you or your
child have previously had an endoscopy, but the number of eosinophils was not
quantified (counted), you are still able to ask the doctor to have the biopsy
slides reviewed and have them counted.
Even if the biopsies are several years old, they can be pulled from
storage (they are archived) and the can be reviewed / re-read. The report can then be amended to indicate
the eosinophil count, even if it is zero.
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Treatment: There is NO cure for Eosinophilic
Esophagitis, but the goal of treatment is to eliminate the eosinophils in the
esophagus, thereby alleviating symptoms.
The treatments include dietary restrictions and medications. Since EoE is usually food-driven
(caused by a reaction to certain foods), most kids and adults with EoE
respond well to dietary treatments, and this may be all that is needed for
many people. However, once treatment
is discontinued, the eosinophils along with the damage they do, will
return. Treatment needs to be ongoing,
which may mean continuing to avoiding the food triggers and/or taking
medication. Elimination
Diet: Dietary restrictions are guided by food
allergy testing (skin prick testing, RAST, and patch testing). Some doctors are recommending that the top
8 allergens be removed from the diet, in addition to the foods that were
identified via allergy testing. The
top 8 allergens include milk, egg,
peanut, tree nut, soy, wheat, fish, & shellfish. Beef is also a common trigger of EoE, as
are certain other foods. However, a
person with EoE can be reacting to any food or combination of foods. While allergy tests are used to
guide an elimination diet, there can be false negative and false positive
test results. You only have to eat one
food that was a “false negative” in an allergy test for the elimination diet
to fail. When an elimination diet does
not do enough to clear the GI tract of eosinophils (as evidenced by scope
with biopsy), sometimes a stricter diet is needed. This may mean just removing some
additional foods from your diet, or going directly to an elemental diet. Food trials can
begin once the symptoms have resolved and the eosinophils are gone,
as confirmed by a clear scope. They
involve adding back one food ingredient
at a time, looking for a reaction, to determine which specific foods are
causing a reaction. Typically, one
single food ingredient is trialed for a 2 week time, looking for a reaction. Some Drs vary the time period by patient
(1-3 wks, typically). If no reaction
is seen, another food can be added and the same time period applies. Usually, when 3-5 foods are added
back, the person has another endoscopy and the Dr looks to see if the
eosinophils have returned. If not, the
foods are ok for that person to eat and they can move on to additional food
trials. If the scope shows that the
eosinophils have returned, all of the foods that have just been trialed must
be removed from the diet once again.
It could be that the person is reacting to only one of the foods, but
without noticeable symptoms to confirm which one, they must all be
suspected. Some people need to scope
between fewer foods. Some even have
even found that they need to trial only one food per scope, but this is not
the norm. Elemental
diet consists of a medical food (elemental formula), without any
proteins, either in its whole or incomplete form (pre-digested or
hydrolyzed). Elemental formulas are
made of amino acids (the building blocks of proteins), fats, sugars, vitamins
and minerals. Amino acids do not cause allergic reactions but whole or
partial proteins can. Medications for Eosinophilic Esophagitis most commonly include
steroids to control inflammation and suppress the eosinophils. Steroids are
used if dietary changes do not resolve the symptoms. They can be taken orally
or topically (swallowed from an asthma inhaler). Side effects from steroids often limit
long-term use of oral steroids.
Without removing the cause of the symptoms via dietary restrictions,
the eosinophils will return once the medication is discontinued. The doctor will determine which, if any,
medications are appropriate for each individual. |
For
additional, detailed information about Eosinophilic Esophagitis,
please visit APFED’s E-Learning Center at
http://apfed.org/drupal/drupal/e-learning-center.
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****DISCLAIMER**** This website has been set up by the parent of a
child with an eosinophilic disorder, not a doctor. The information shared on this website is
not intended to replace |