Eosinophilic Esophagitis (when acid reflux is not reflux) by Lester Stine, MD

In your blood stream, there are three basic types of blood cells: red blood cells which carry oxygen, platlets which help clot your blood when you get a cut, and white blood cells which have several functions. One special type of white blood cell, called the eosinophil, has been associated with allergic reactions. The normal esophagus does not contain eosinophils. However, these cells may be found in the esophagus in a number of diseases, most notably acid reflux, or more formally termed gastroesophageal reflux disease (GERD).

Beginning in the early 1970's it was noted that some patients who presented with unresponsive acid reflux or with difficulty swallowing (dysphagia) occasionally had multiple esophageal rings (areas of narrowing) upon upper GI endoscopy. Some of these patients were found to have increased numbers of eosinophils in the esophagus on biopsy specimens and were diagnosed with "Eosinophilic Esophagitis."

Is Eosinophilic Esophagitis common?
Eosinophilic Esophagitis is not common. In fact, in the past, it was considered to be a rare occurence and mostly a disease of children. Recently however, there have been an increased number of cases involving adults. It was not described before the 1970's. It now is known to affect both children and adults. Males are affected more commonly than females, and it is more common in younger (20s to 30s) that older adults. The typical patient complaints of difficulty swallowing, which may have been present for years. They may also have symptoms of heartburn and acid reflux which have not responded to medications nor antireflux surgery. Children can complain of vomiting, feeding disorders or abdominal pain. Often times there is a history of other allergic diseases such as asthma, food or environmental allergies, hives, etc. Of note, there is no known increased incidence of cancer in the esophagus in patients with this condition.

How is Eosinophilic Esophagitis diagnosed?
The diagnosis of Eosinophilic Esophagitis is typically made by endoscopy. There are characteristic features of the esophagus including multiple rings, furrowing ("train track sign") and diffuse narrowing of the esophagus which can suggest the diagnosis. However, the diagnosis typically requires biopsy of the esophagus. The biopsy specimens show an increased number of eosinophils in the esophagus, although the exact number required to make the diagnosis is not known.

What about treatment?
The best way to treat this disease is not well know, since the exact cause is not understood. Various treatments have been tried including:

• 1. Acid suppression/treatment for reflux
  A trial of a Proton Pump Inhibitor (for example Prilosec) may be useful, although most patients will not respond. There are occasional patients who have acid reflux as their main problem or as a coexisting problem and those people will be helped with these medications.

• 2. Esophageal dilatation
  Often patients with difficulty swallowing and evidence of narrowing of the esophagus will undergo dilatation of the esophagus with special dilators that get progressively larger, and therefore stretch the esophagus or with balloons passed through an endoscope that are inflated to various sizes to stretch the esophagus. In patients with Eosinophilic Esophagitis there is an increased incidence of deep esophageal tears or perforation (full thickness tears in the esophagus resulting in a rupture.) Therefore care must be taken when dilating a patient with Eosinophilic Esophagitis. A tear in the esophagus can require a stay in the hospital, and may require surgery.

• 3. Elimination diets
  If Eosinophilic Esophagitis is caused by an allergic reaction, it makes sense to try to eliminate the food causing the allergy. This, however, can be difficult. Allergy testing for food may not be accurate, the number of foods tested for is relatively small, and diets may be difficult to follow. Also, the long term success of this approach has not been well studied. However, this may be worth a try in patients with this disease as it is the most natural approach. Also, over time, certain food may be reintroduced into the diet without further problems. Some studies suggest it is not ingested, but inhaled substances that cause this disease.

• 4. Low Residue Diets
  If difficulty swallowing persists, patients are usually advised to emphasize softer foods in their diet. They must be careful to always chew well and drink plenty of water with meals. They should avoid tough meat, fresh fruit and vegetables (especially with skins), doughy bread products, clumpy rice, or any food that has given them problems in the past.

• 5. Steroids
  Steroids are powerful medications than can reduce inflammation and treat allergies. They can be taken in pill form, such as prednisone, and have shown good results in treating this disorder. However, when the pills are stopped the symptoms will typically return. Also, oral steroids can have significant long term side effects.

  Steroids can also be administered using an inhaler. Inhaled steroid sprays have also shown promise in patients with asthma. At present, there is no FDA-approved steroid spray to treat Eosinophilic Esophagitis. However, there have been many reports showing that a common asthma inhaler called Fluticasone (Flonase) can be used for this purpose. Rather than breathing in the inhaled spray, the patient swallows it allowing it to directly coat the lining of the esophagus. In this manner less steroid is absorbed into the bloodstream and side effects are much less than prednisone pills.

  Summary
  In summary, Eosinophilic Esophagitis is an uncommon disorder of the esophagus characterized by increased numbers of eosinophils in the lining of the esophagus. It is often confused with acid reflux, but typically, does not respond to standard anti-reflux therapy. It ususally presents with difficulty swallowing with or without heartburn. The cause is not known, but an allergic response is suspected. Treatment options are limited and described above. Hopefully, as more and more cases are reported, better medications will be discovered.

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