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Eosinophilic esophagitis

Basics

The eosinophilic Esophagitis is an allergic esophageal inflammation: the asthma of the esophagus. It is named after the observation that a special type of inflammatory cell, the eosinophil granulocyte (eosinophil), enriches within the esophagus.
Eosinophil granulocytes (eosinophils) are immune cells, which play an important role in the mediation of allergy. The cells are named due to their eosinophilic staining characteristic. The term “eosinophils” comes from the observation, that the cells enrich the red dye eosin during a routine stain with hematoxylin and eosin (greek phi-lein: love; eos: dawn; eosin: name of the red dye). Consequently the eosinophil granulozytes appear as pale red cells when examined under the microscope.
The eosinophilic Esophagitis has first been described in 1978. It affects children and adolescents and increased in the last 10-20 years.

Causes

The exact cause for eosinophilic Esophagitis (EoE) has not yet been defined. It is the asthma of the esophagus. Consequently food intolerance and allergies play a role in the development of the disease.
A complex allergic type immune response drives the inflammation. The most inner layer ( Mucosa) of the esophagus orchestrates an allergic reaction. This allergy response traffics eosinophils into the Mucosa. The inflammation only involves the squamous lined esophagus. It does not affect the columnar lined esophagus (=the Reflux-induced Mucosa of the esophagus). In addition, the EoE shows a patchy distribution. The intensity of the inflammation occurs along a gradient, which increases from the lower end to the upper end. Therefore, the inflammation and eosinophils concentrate towards the upper end of the esophagus.

Symptoms

Symptoms of include eosinophilic Esophagitis (EoE) include swallowing difficulties ( Dysphagia), Heartburn, chest pain, nausea and vomiting. In 50% of the cases the patients present in the emergency room with Dysphagia and food impaction. However, none of the symptoms is specific for EoE.
Similar symptoms occur in Gastroesophageal reflux disease ( GERD) and other functional disorders of the esophagus ( Achalasia, diffuse esophageal spasm). Finally these findings may indicate a systemic disease including sclerodema or lupus erythematodes.
When children develop food refusal and feeding intolerance this could be an indicator for EoE.

Diagnosis and tests

Diagnosis of eosinophilic Esophagitis is established by Gastroscopy (esophagogastroduodenoscopy). Typical endoscopic findings include rings, lines and the swelling within the inner most layer of the esophagus. The tissue is highly vulnerable and tends to bleed when touched by the endoscope. Biopsies (tissue samples) are obtained from the upper and lower end of the esophagus. The pathologist establishes the diagnosis during the microscopic examination of the esophageal biopsies. The presence of more than 15 eosinophil granulocytes per high power field defines the eosinophilic Esophagitis (EoE). In addition microscopy may reveal micro-abscess formation and edema (=swelling of the tissue).

Allergy tests specify the type of food and drug intolerance.

In 10%-20% of the cases EoE associates with Reflux. In these cases we perform Esophageal manometry and Reflux monitoring.

Normal

Schematic drawing of the microscopic image of a tissue sample of a person with eosinophilic Esophagitis (EoE). Note the increased number of eosinophils (more than 15 per high power field) within the normal Squamous epithelium (yellow cells). Basal lamina is a specialized tissue scaffold for the epithelial cells (yellow cells).

Treatment

Treatment of eosinophilic Esophagitis includes the peroral administration of proton pump inhibitor (twice 40 mg daily) and cortison spray. Based on the data obtained by the allergy test, the diet eliminates allergenic nutrients (mainly sugars: lactose, fructose). For children the medical therapy has to be adjusted according to the bod weight (10-20 mg proton pump inhibitor daily).
Reflux is treated either by medical therapy or Anti reflux surgery. Feel free to contact us for more information.
Impacted food is removed during an Endoscopy. Stenosis is treated by endoscopic balloon dilatation.

Prevention

Prevention avoids allergenic food and drugs.

Self test

There exists no self test for eosinophilic Esophagitis. In case of food impaction, one should immediately consult the emergency unit of the neighboring hospital for endoscopic removal.

Expert opinion

Johannes Lenglinger (Physiologist, Vienna):

Eosinophilic Esophagitis increases in frequency. And it parallels the increase of allergic diseases in general. This may be due to the life style and the eating behavior. The typical symptom is the food impaction. Here we recommend an urgent Endoscopy for the removal of the food. Thereafter we do Endoscopy and Biopsy sampling. The pathologist establishes the diagnosis. Here we commence a first line treatment with proton pump inhibitor and cortison spray. If Reflux is suggested as an additional cause for the disease, we perform Esophageal manometry and Reflux monitoring.

Martin Riegler (Surgeon, Vienna):

The eosinophilic Esophagitis is the asthma of the esophagus. Allergies and food intolerances play a major role in the development of the disease. Children with this disease refuse to eat and develop other symptoms of allergy (cough, wheezing, asthma). Mothers should get alert and let them be seen by a physician.

Fritz Wrba (Pathologist, Vienna):

The pathologist examines the esophageal biopsies under the microscope. If the number of eosinophils per high power field equals more than 15, we assess the diagnosis of eosinophilic Esophagitis. However, without obtaining the right biopsies we cannot establish the diagnosis. Therefore it is essential that the surgeon or gastroenterologist obtains 4-5 biopsies form the upper and the lower end of the esophagus, respectively. The biopsies from the different locations should be processed in separate beakers.

Literature

  1. Landres RT, Kuster GGR, Strum WB. Eosinophilic Esophagitis in a patient with vigorous Achalasia. Gastroenterology 74: 1298-1301.
  2. Odze RD. pathology of eosinophilic Esophagitis: what the clinician needs to know. Am J Gastroenterol 2009; 104: 485-90.
  3. Dellon ES, Gibbs WB, Fritchie KJ et al. Clinical, endoscopic, and histologic findings distinguish eosinophilic Esophagitis
  4. from Gastroesophageal reflux disease. Clin Gastroenterol Hepatol 2009; 7(12): 1305-13.
  5. Dellon ES. Eosinophilic Esophagitis: diagnostic tests and criteria. Curr Opinion Gastroenterol 2012: 28.
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