Eosinophilic Esophagitis

What is allergic esophagus and eosinophilic esophagitis?
Allergic esophagus is the lay term for a condition known as eosinophilic
esophagitis, abbreviated EE or EoE. It is characterized by inflammation of the
esophagus or feeding tube resulting from a type of white blood cell called the
eosinophil. This reddish appearing cell is typically found in tissues of the
body affected by allergic reactions and parasite infections. The cell was named
by Paul Erlich in 1879 after Eos, the Greek goddess of dawn. When activated
eosinophils release chemicals like histamine that can cause intense swelling,
itching, and damage tissues.
What are the symptoms of eosinophilic esophagitis in adults?
In adults eosinophilic esophagitis usually causes intermittent episodes of food
sticking. Food sticking or hanging up after swallowing is called dysphagia. In
classic eosinophilic esophagitis a young man or teenage boy presents with
episodes of food being lodged or stuck in the esophagus or feeding tube. Food
that won’t go down and can’t be regurgitated up in the background of a history
of difficulty swallowing foods such as bread or dry meats like chicken and beef
is a common history. When associated with a personal and/or family history of
allergic conditions eosinophilic esophagitis is highly likely.
How does eosinophilic esophagitis present in children?
Young children with eosinophilic esophagitis may present with complaints of
chest pain, abdominal pain, poor appetite, regurgitation or reflux, vomiting, or
failure to grow (failure to thrive) normally. Some may have unexplained low
blood counts or iron deficiency from bleeding from the esophagus.
How is eosinophilic or allergic esophagitis diagnosed?
It is suspected by the classic history and the appearance of the esophagus on
endoscopy. Eosinophilic esophagitis typically results in the esophagus having
multiple rings or constrictions that result in the appearance of a cat’s
esophagus. This is called felinization of the esophagus or ringed esophagus.
Other visual signs may be whitish spots, long furrows or a lining that looks
like crepe paper and is very easily torn.
What are the diagnostic microscopic criteria for eosinophilic
esophagitis?
The definitive diagnosis is made by the finding of abnormal numbers of
eosinophils in esophageal biopsy tissue. The esophagus normally contains no
eosinophils. The number of eosinophils considered abnormal is debated. However,
between >15-25 eosinophils per high power field (400x) is considered abnormal
with most authorities agreeing that >20 eosinophils is diagnostic. It has been
long accepted that chronic acid reflux is commonly associated with 5-10
eosinophils per high power field. However, these eosinophils should be found
only in the lower esophagus. Finding eosinophils higher in the esophagus greatly
increases the likelihood that eosinophilic esophagitis is present.
Why is it important to distinguish eosinophilic esophagitis from acid
reflux esophagitis?
Acid reflux esophagitis usually responds to acid blocker medications like
histamine 2 blockers ranitidine (Zantac), cimetidine (Tagamet), famotidine
(Pepcid) and proton pump inhibitors like omeprazole (Prilosec) or esomeprazole
(Nexium). However, though symptoms like heartburn associated with eosinophilic
esophagitis may improve with these medications, the swallowing difficulties and
chest pain do not.
How are the swallowing difficulties different in reflux and eosinophilic
esophagitis?
Acid reflux injury to the esophagus often results in narrowing or constriction
of the lower esophagus that causes a food- sticking sensation. Esophageal
strictures due to acid reflux are treated by a procedure known as esophageal
dilation to stretch the constriction to allow normal swallowing. In the setting
of acid reflux, esophageal dilation is usually safe and highly effective.
Eosinophilic esophagitis usually results in swallowing difficulties. However,
the constrictions of the esophagus that occur in eosinophilic esophagitis are
usually multiple rings located higher in the esophagus. Because they are due to
injury to the esophagus from the release of allergic chemicals these rings carry
a high risk of tearing the esophagus if dilation is attempted before treatment
with steroids.
Is eosinophilic esophagitis associated with an increase risk of
esophageal cancer?
There does not seem to be an association of cancer of the esophagus with
eosinophilic esophagitis like there is with chronic acid reflux. However,
eosinophilic esophagitis can be chronic and difficult to treat.
How is eosinophilic esophagitis treated?
Identification and elimination of problem allergy foods is the mainstay of
treatment. Foods that are associated with positive allergy blood tests or skin
tests are eliminated. In some people a strict elimination diet is required.
Rarely, a diet of only basic amino acid proteins in a liquid (elemental diet) is
required. Temporary relief can be achieved with steroids. Systemic (oral
prednisone) works but has the potential side effects of steroids on the rest of
the body. Topical steroids, steroid applied directly to the surface that has
little or no absorption into the blood stream is preferable. The nasal steroid,
fluticasone propionate, has been used successfully. It is sprayed in the mouth
and swallowed twice daily. The mouth should be rinsed out followed by spitting
out rather than swallowing the water. No eating or drinking for 30 minutes is
recommended. A few studies have reported response to mast cell stabilizer
disodium cromoglycate (Cromolyn), leukotriene inhibitor montelukast (Singulair),
immunomodulators such as aziothioprine (Imuran), or monoclonal antibody against
IL-5 mepolizumab.
What role do food allergies play in the treatment of eosinophilic
esophagitis?
Food allergy plays a major role in the cause and treatment of eosinophilic
esophagitis, hence the alternative term used, allergic esophagitis. Most
patients with eosinophilic esophagitis will be found to have one more food
allergies when adequate testing is done. Skin prick allergy testing or blood
tests (RAST, IgE food antibodies) can be negative but patch skin testing or
intradermal testing may be positive. Sometimes, a strict elimination diet with
re-challenge is the only way to implicate a problem food. In most patients a
personal and family history of allergic disorders (atopy) such as allergic
rhinitis (hayfever), asthma, eczema, atopic dermatitis, or food allergies is
present.
What are the most problem common foods associated with eosinophilic
esophagitis?
In descending order, the most common foods reported in the largest
series are milk, egg, soy, corn, wheat, beef, chicken, potato, oats, peanuts,
turkey, barley, pork, rice, green beans, apples, and pineapple.
What is the role of elimination diet in eosinophilic esophagitis?
Elimination diet is the most effective treatment in eosinophilic
esophagitis. However, elimination diets are difficult to follow for both adults
and children and strict elimination diets are very difficult if not impossible
to follow long term. Recently, Kagalwalla et al. published their success with a
six-food elimination diet (SFED). The SFED restricts foods from six of the most
common food allergen categories. The SFED eliminates cow’s milk protein
(casein), soy, wheat, egg, peanut/tree nuts, and seafood. When compared with an
elemental diet (ELED) the SFED is not quite as effective (74% versus 88%
achieving significant improvement) but has better acceptance, cost and
likelihood of compliance. Processed foods containing any known or suspected
foods must also be eliminated during an elimination diet.