Scot M. Lewey, DO, FACG, FASGE, AGAF
Clinical Professor of Medicine
Eosinophilic esophagitis, abbreviated EE or EoE, is an inflammation of the esophagus or feeding tube characterized by the presence of a type of white blood cell called eosinophil. It is a reddish appearing cell that is typically found in tissues of the body affected by allergic reactions and some parasite infections. The cell was named by Paul Erlich in 1879 after Eos, the Greek goddess of dawn. It is also called allergic esophagitis. Eosinophils, when activated, release chemicals that can cause intense swelling, itching, and tissue damage.
The symptoms of EoE in adults is classically episodes of food sticking when swallowing that is called dysphagia in medical terms. Typically, a young man or teenage boy presents with an episode of food being lodged in the esophagus or feeding tube. Food that won't go down and can't be regurgitated up along with a history of difficulty swallowing foods (such as bread or dry meats like chicken and beef) is common, especially associated with a personal and/or family history of allergic conditions.
Failure of the food to go down (or up) causes a food impaction resulting in the inability to swallow even saliva, chest discomfort and usually sends the person to the emergency room. Sometimes, intravenous medication that relaxes the esophagus will allow the food to pass but usually it has to be removed by an endoscope. The classic patient has little or no heartburn though some people have moderate to severe heartburn symptoms. The tip off in this setting is that the heartburn symptoms frequently don't improve with acid blocking medications.
Young children may present with complaints of chest pain, abdominal pain, poor appetite, regurgitation or reflux, vomiting, or failure to grow (failure to thrive).
Yes, sometimes there are little symptoms but it is found during endoscopy, especially it may present as unexplained low blood counts or iron deficiency from bleeding from the esophagus.
It is first suspected by the classic history and the appearance of the esophagus on endoscopy of multiple rings or constrictions that result in the esophagus looking like that 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.
The definitive diagnosis is made by the finding of an abnormal number of eosinophils in esophageal biopsy tissue. The number of eosinophils considered abnormal is debated between >15-25 eosinophils per high power field (400x) with most authorities agreeing >20 as being diagnostic. The esophagus normally contains no eosinophils. It has been long accepted that chronic acid reflux commonly can be associated with 5-10 eosinophils per high power field in the lower esophagus but this finding higher in the esophagus should raise likelihood that eosinophilic esophagitis is present.
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) whereas eosinophilic esophagitis often does not get better with these medications. Acid reflux injury to the esophagus can result in narrowing or constriction of the lower esophagus causing a food sticking sensation. This can be treated by a stretching of the constriction known as esophageal dilation that is usually safe and highly effective. Eosinophilic esophagitis also may result in constrictions of the esophagus but the stricture or rings are usually multiple, located higher in the esophagus and carry a high risk of tearing or puncturing the esophagus if dilation is attempted before treatment with steroids. There does not seem to be an association of cancer of the esophagus with eosinophilic esophagitis like there is with acid reflux. However, eosinophilic esophagitis can be chronic and difficult to treat.
Identification and elimination of problem allergy foods is the mainstay of treatment. Foods that show positive allergy testing are eliminated. In some people a strict elimination diet is recommended. 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 applied directly to the surface that have little or no absorption into the blood stream are 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 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.
Food allergy plays a major role in the cause and treatment of eosinophilic esophagitis, hence the alternative term used allergic esophagitis. Most patients will be found to have one or 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, elimination diet with re-challenge is the only way to implicate a problem food. In most patients a personal and/or family history of allergic disorders (atopy) such as allergic rhinitis (hayfever), asthma, eczema, atopic dermatitis, or food allergies is noted.
In descending order, the most common foods reported in the largest series were, milk, egg, soy, corn, wheat, beef, chicken, potato, oats, peanuts, turkey, barley, pork, rice, green beans, apples, and pineapple. Elimination of foods that test positive on allergy testing is the cornerstone of treatment of eosinophilic esophagitis. However, sometimes allergy testing is negative or inconclusive. It it then that an elimination diet is necessary and a pre-elimination diet food symptom diet diary is very helpful.