Eosinophilic esophagitis (EE) is an allergic inflammatory disease of the esophagus (the tube that connects the throat and the stomach). It is a newly diagnosed and not fully understood disease. Some of the symptoms of EE mimic those of gastroesophageal reflux (GERD). EE is usually associated with food or environmental allergens and is most often seen in children.
An increasing number of EE cases have been diagnosed within the last several years. It is not fully known why the disease occurs most often in children, or why more cases of EE are being diagnosed.
EE causes abdominal pain, very painful swallowing and digestion, and vomiting. It can result in children failing to thrive and weight loss. There is a slight predominance of the disease in males.
EE may be treated by medications typically used to treat asthma, such as corticosteroids. Other asthma medications, like bronchodilators, should not be used to treat eosinophilic esophagitis as they may increase the stomach acid reflux into the esophagus. Medications traditionally used to treat heartburn (e.g. antacids) are not normally effective with EE.
EE can be treated by avoiding the allergen causing the reaction. In the case of food allergy, the preferred treatment method is strict avoidance of the problem food. An elimination diet may need to be undertaken to identify the particular food(s) causing the allergy and a special diet may need to be developed for the long term. An environmental allergy may be treated by a combination of avoidance and medications such as antihistamines.
About eosinophilic esophagitis (EE)
Eosinophilic esophagitis (EE) is an allergic inflammatory reaction of the esophagus (the tube that connects the throat and the stomach) which causes vomiting, and painful swallowing and digestion. Also known as allergic esophagitis, EE is a newly diagnosed disease whose symptoms mimic some of those associated with gastroesophageal reflux disease (GERD).
EE is caused by a large build up of eosinophils in the esophagus. Eosinophils are a type of white blood cell. Designed to respond to parasites, eosinophils are normally found in the intestines and are not supposed to be present in the esophagus.
Although the disease occurs in adults, most cases of EE have developed in children. It is not fully known why the disease occurs more often in children. EE occurs more commonly in boys than in girls. In comparison, most adults affected by the condition are men in their 20s and 30s.
Incidence of EE is on the rise, with an increasing number of EE cases having been diagnosed within the last five years. Physicians do not completely understand whether EE is occurring more often or if it is just being diagnosed more frequently.
The origin of EE is an allergic reaction, which involves the immune system’s inappropriate response to a protein. The body perceives the protein to be harmful, and produces immunoglobulin E (IgE) antibodies to defend against the "invader." When the IgE antibodies come in contact with the offending protein, chemicals known as histamines and leukotrienes are released, causing the symptoms most people associate with allergies, including itchiness, rashes, hives, swelling, nausea, diarrhea and shortness of breath.
Food allergies such as milk, eggs, nuts, wheat, soy, corn and shellfish are the cause of 80 percent of EE, according to the American Academy of Allergy, Asthma and Immunology (AAAAI). Environmental allergens, such as pollen and mold, are also related to EE. The allergic reaction creates inflammation in the esophagus which causes vomiting, difficulty swallowing, nausea and abdominal pain in susceptible individuals.
EE may be misdiagnosed as GERD as some of the symptoms are similar, and GERD is common. However, symptoms of EE do not improve with acid blockage medications like those used to treat GERD. EE does appear to improve with an elimination diet to remove the food allergen source or with corticosteroid medications commonly used to treat asthma.
Left untreated, the inflammation related to EE will begin to damage the esophagus, resulting in narrowing of the esophagus (stricture) and in increase in fibrous tissue (fibrosis).
Related conditions to eosinophilic esophagitis
Conditions related to eosinophilic esophagitis (EE) include:
Gastroesophageal reflux disease (GERD). A condition that causes stomach acid to back up into the esophagus, causing heartburn and other symptoms. Although the symptoms of EE and GERD are similar, the two conditions differ. Unlike GERD the symptoms of EE do not respond to medications such as antacids.
Eosinophilic gastroenteritis. An uncommon disease where eosinophils are found in the gastrointestinal tract.
Eosinophilic colitis. An uncommon disease where eosinophils are found in the large intestine.
Potential causes of eosinophilic esophagitis
Some of the potential causes of eosinophilic esophagitis (EE) include:
Food allergies. Involves a response by the immune system to a specific food or food component causing symptoms such as itchiness, rashes, hives, swelling, nausea, diarrhea and shortness of breath. More severe reactions to food may result in potentially life-threatening anaphylaxis or anaphylactic shock. Most people with food allergies are allergic to eggs, cow’s milk, soy, wheat, corn, peanuts, tree nuts, fish and shellfish. These are also the food allergens associated with EE.
Asthma. A chronic inflammation of the body’s bronchial (airway) tissues that afflicts millions of people in the United States. People with asthma experience shortness of breath, chest tightness, coughing and wheezing. These symptoms intensify during an asthma attack, which occurs when exposure to allergens or other stimuli further inflame the airways, leading to an inability to expel trapped air from the lungs. New research indicates that eosinophils are required for the mucus accumulation and lung problems associated with asthma. The presence of eosinophils and the continuum of the respiratory and gastrointestinal tracts are believed to be the link between asthma and EE.
Allergic rhinitis. Commonly called hay fever, allergic rhinitis is an inflammation of the inner lining of the nose that occurs when an allergic individual encounters an airborne allergen such as pollen, mold, dust mites or animal dander. Usually inhaled, these triggers generate allergy symptoms such as sneezing, coughing, runny nose, sore throat, anditchy and runny eyes. Allergic rhinitis is very common, with an estimated 20 percent of Americans suffering from the condition, according to the American College of Allergy, Asthma and Immunology.
Other factors. There has been a dramatic increase in rates of EE since 2000. Researchers at the Cincinnati Children’s Hospital studying EE found that of the 315 cases since 1991, only eight children had the characteristic symptoms of EE prior to 2000. The occurrences of this disease have increased so much in recent years that they may now be at higher levels than those for Crohn’s disease or ulcerative colitis. Some speculate that the “hygiene hypothesis” (the concept that children are not exposed to sufficient germs or bacteria to permit full development of the immune system and are therefore at an increased risk of many immune system related disorders) may be a factor.
Signs and symptoms of EE
The signs and symptoms of eosinophilic esophagitis include:
Difficulty swallowing (dysphagia)
Heartburn
Nausea
Vomiting
Chest pain
Discomfort behind the sternum
Abdominal pain
Food impaction
Choking
Regurgitation
Cramping
Diarrhea
Difficulty breathing
Failure to thrive
Weight loss
Diagnosis methods for eosinophilic esophagitis
Because the symptoms of eosinophilic esophagitis (EE) mimic those of medical conditions that are far more common, a number of conditions may need to be ruled out as the cause of the patient’s symptoms. The physician will develop a detailed medical history which will include any incidents of GERD, family history of food allergy, asthma, allergic rhinitis and gastrointestinal disorders.
If food allergy is determined to be a likely source of symptoms, a physician will create a dietary history of the patient. The history may include:
Timing of the reaction (i.e., whether the reaction occurred within an hour after eating)
Whether the reaction is always associated with a certain food
How much of the food was consumed, because the severity of a reaction may relate to the amount of food consumed
How the food was prepared (i.e., was it consumed raw or thoroughly cooked)
Whether treatment for a reaction was successful (i.e., if taking antihistamines relieved the symptoms)
Although these first steps are often inconclusive, they can create a better idea of where to proceed with further testing. Tests to identify a specific food allergen include the elimination diet and oral food challenge.
Physicians can also perform skin testing to narrow the cause of symptoms to a specific allergen. During skin testing the physician will prick or inject allergen extracts into the patient’s skin. The development of a small, raised, reddish area indicates a positive reaction.
Skin testing is not practical for those with a skin condition. In such cases, a blood test such as a radioallergosorbent test (RAST) may be the best way to measure an allergic reaction. This type of blood test allows a laboratory to directly test a blood sample for antibodies that correspond to specific allergens. Patients with EE may have elevated levels of IgE in their blood.
The physician may also want to examine the esophagus itself. In this circumstance an endoscopy is performed to show the conditions inside the esophagus.
During an endoscopy, a special telescope is inserted into the nose, sinus cavities or the throat to allow the physician to examine those areas. Biopsies (removal of tissue from a body area for examination) can be taken with an endoscope which can determine the actual amount of eosinophils in the esophagus. An endoscopy may involve restricting the patient’s diet in preparation for viewing the esophagus prior to the procedure and the administration of a sedative during the procedure.
Treatment options for eosinophilic esophagitis
Eosinophilic esophagitis (EE) originates from an allergic reaction. Once an allergic reaction has occurred, treatment is focused on relieving symptoms. There are a variety of medications that may be recommended depending on the reaction.
Medications like antihistamines provide relief for common allergic reactions such as hives, sneezing, runny nose and gastrointestinal symptoms. Antihistamines directly counteract the effects of the histamines, which are responsible for most allergy symptoms. With mild symptoms, these drugs are usually administered orally. For more severe allergic reactions, a physician may recommend an injected form of antihistamine.
Corticosteroids are medications that reduce inflammation. These medications have been successfully used to treat EE in some patients.
Bronchodilator medications can be used to treat asthma but should not be used to treat EE. Research indicates that bronchodilator drugs may increase the reflux of acid into the esophagus, making the condition worse.
Prevention methods for EE
Eosinophilic esophagitis (EE) is caused by an allergic reaction that occurs in the esophagus, most often to particular foods. The most effective way to prevent any allergic reaction is to avoid the allergen that triggers the reaction. Consultation with a physician will determine what type of allergy is causing the symptoms and the physician will advise the patient on methods to avoid the allergen.
There are currently no drugs available that can prevent a food allergy from taking place. The most fundamental and effective form of prevention is the complete removal of problem foods from the diet.
Ongoing research regarding EE
The latest ongoing research involving eosinophilic esophagitis (EE) includes:
Drug research. New research has focused on controlling the creation of cytokines. A cytokine is a hormone–like protein that is produced by white blood cells and acts as a messenger between cells. IL-5 is a cytokine involved in regulating the production, activation and tissue recruitment of eosinophils. Anti-IL-5 drugs are being developed that may be helpful in treating eosinophilic esophagitis. Anti-IL-5 drugs are administered intravenously.
Genetics. Research suggests that a gene known as eotaxin-3 may play a role in the development of EE. Further research may lead to the development of new treatment methods, including a drug that blocks the gene.
“One airway” disease and the development of “total airway” specialists. Many people with asthma, allergic rhinitis or allergic asthma see different specialists for treatment and prevention. Recent research has indicated that the relationship between the upper and lower airways is strong and similar to a continuum. Links between asthma, allergies and gastrointestinal complaints may also be related to the continuum between the airways. The concept behind “total airway” specialists would reflect the relationship between the airways within the body and coordinate the professional knowledge, diagnostic methods and treatment of these related conditions.
Allergies. There is a worldwide increase in new allergic-inflammation based disorders. Eosinophilic esophagitis is an emerging disease throughout the world with newly documented cases appearing in England, Japan, Spain, Australia, Switzerland and Italy. Research is ongoing to identify possible connections between diseases and new medications to treat these conditions.
Questions for your doctor
Preparing questions in advance can help parents to have more meaningful discussions with their child’s physicians. Parents may wish to ask child’s doctor the following questions related to eosinophilic esophagitis (EE):
Do my child’s symptoms suggest EE?
What methods will you use to determine if my child has EE?
Is EE dangerous to my child’s overall health?
What is causing my child’s EE?
Do you have experience treating this condition, or should I seek a specialist?
What are my child’s treatment options?
Will my child have to take medication for the rest of his/her life?
How can I prevent my child from having reactions in the future?
Will my child outgrow the condition?
Are my other children more likely to develop EE because their sibling has it?