What is Eosinophilic Esophagitis?
The esophagus, the tube that connects your throat to your stomach, doesn’t naturally contain cells called eosinophils. So, if these cells are present, it signals that something is not right. At one point, eosinophilic esophagitis – a condition where eosinophils show up in the esophagus – was believed to be part of acid reflux disease. But now we know it’s a separate health issue as we learn more about how the esophagus interacts with the immune system.
Eosinophils appear in the esophagus in reaction to different triggers or antigens – substances that the immune system responds to. Eosinophilic esophagitis (EoE) is a long-lasting condition where the esophagus reacts to an antigen in the body, and clinically it presents as various esophagus-related problems. When looked at under the microscope, inflammation spearheaded by eosinophils is noted, but it only involves the esophagus.
Before a diagnosis of EoE is confirmed, it’s important to check for and rule out other diseases that can also cause an increase in eosinophils.
What Causes Eosinophilic Esophagitis?
The exact cause of Eosinophilic Esophagitis (EoE), a condition that affects the tube connecting your mouth to your stomach, is unknown. It’s thought to be due to a combination of factors including one’s environment, genes, and immune system. It’s possible that an allergy to certain foods could lead to EoE, but it’s rare for these patients to have severe allergic reactions to food.
There’s a strong connection between EoE and atopy, a tendency to be extra sensitive to certain allergens. This means people with EoE often also have other related conditions. They might have a history of prolonged seasonal allergies, asthma, atopic dermatitis (a type of skin inflammation commonly known as eczema), or other conditions related to allergies or the immune system.
Risk Factors and Frequency for Eosinophilic Esophagitis
EoE, or eosinophilic esophagitis, is a condition that affects both children and adults all around the world, except Africa. It appears more often among boys in the younger age group, while among adults, white men, specifically those who are not Hispanic, are more likely to have it than women. The highest occurrence is among men in their 20s and 30s, and on average, people are diagnosed at age 34.
- EoE is common in children and adults.
- It has been reported in all continents except Africa.
- Worldwide, the incidence of EoE is between 0.1/10,000 to 1.2/10,000.
- In children, EoE is more common in boys.
- In adults, it’s more likely to be diagnosed in Caucasian, non-Hispanic men, at 76% compared to 48% in women of the same races.
- Although EoE can affect all age groups, it’s most common in men in their 20s and 30s.
- The average age of diagnosis is 34.
Signs and Symptoms of Eosinophilic Esophagitis
Eosinophilic esophagitis (EoE) diagnosis takes a close look at your medical history due to its symptoms being quite similar to gastroesophageal reflux (GERD). Adults with EoE often experience difficulty swallowing solid food. A common symptom that drives many to the emergency department is food becoming stuck in the esophagus. Other symptoms may include chest pain and heartburn. Children may experience symptoms like nausea, vomiting, not being able to tolerate certain foods, abdominal pain, and weight loss. People with EoE may also have a history of other conditions, like asthma, atopic dermatitis, seasonal allergies, food allergies, allergic rhinitis, and eczema.
A physical examination during the diagnosis is not as vital as looking at one’s medical history. The most typical physical symptom, if any, would be tenderness in the abdomen without any sign of peritonitis (inflammation of the abdominal lining).
Testing for Eosinophilic Esophagitis
The diagnosis of EoE, or Eosinophilic esophagitis, can only be made after several evaluations. If a person has symptoms like food getting stuck in their throat (food impaction), difficulty swallowing (dysphagia), or a history of allergic disorders (atopy), they should get a procedure called an upper endoscopy. This test involves using a slender tube with a light and camera on the end to examine the esophagus, the tube that connects your throat to your stomach.
In this procedure, a small piece of tissue from the esophagus is also collected, a process known as a biopsy. The clinicians can examine this sample under a microscope to help diagnose EoE.
This process should also be done for those whose heartburn does not respond to a type of medication called proton pump inhibitors taken at suitable doses for 8 to 12 weeks, as these individuals could potentially have EoE too. The biopsy should take samples from different sections of the esophagus, including the upper, middle, and lower areas. Additional biopsies may be needed from the stomach and upper part of the small intestine to rule out other conditions contributing to high levels of eosinophils, a type of white blood cell.
Various signs from the endoscopic procedure can suggest EoE; these include a grooved or ridged surface within the esophagus, white patches or fluid buildup, stiff esophageal rings, narrowing of the esophagus, or fragile tissue that tears easily when the endoscope is inserted. However, it’s important to note that some people may have a normal-looking esophagus despite having EoE.
The pathological diagnosis, or more definitive confirmation of EoE, depends on if there are more than 15 eosinophils per high-power microscopical field within the biopsy sample. Other signs suggestive of EoE can include increased basal cells, elongated structures known as papillae, eosinophils in the surface layers, granules released from eosinophils, and fibrosis or thickening of the layer beneath the surface.
Currently, there isn’t a laboratory test specifically to diagnose EoE. However, individuals with EoE often have an elevated level of a specific type of immune cell called IgE. A barium swallow test, which involves swallowing a liquid that aids in visualizing the esophagus on X-rays or scans, could show narrowing or ring-like structures in the esophagus that may suggest EoE.
Finally, if a person has a history of allergic or food-related disorders and is diagnosed with EoE, they should be evaluated by an allergist or immunologist, specialists in allergic diseases, to assist in their care.
Treatment Options for Eosinophilic Esophagitis
The goal in treating EoE, or Eosinophilic Esophagitis, is to manage the symptoms by reducing the number of certain cells called eosinophils in the esophagus. Lowering these cells minimizes inflammation in the esophagus. The treatment plan usually includes changes in diet, medication, and sometimes procedures called endoscopies.
Diet Plan:
For patients who have a history of allergies to specific foods, changes in their diet can help the symptoms of EoE. The plan is to avoid the particular food causing the allergic reactions. However, if it’s not clear what food or substance is causing the problem, doctors might suggest a ‘six food elimination diet’ or SFED. The commonly allergenic foods to stay away from include cow’s milk, wheat, peanuts and tree nuts, eggs, soy, and seafood. Another option is an ‘elemental diet’, which consists of essential amino acids. Some people on this diet may need a tube inserted into their stomach to ensure they receive enough calories. Studies have shown that this more restrictive elemental diet can be more effective. It’s generally advised that the success of the diet is measured by how well the symptoms are managed and by using an endoscopy to check whether the number of eosinophils have decreased.
Endoscopy should be performed every time a food is added back or removed from the diet to examine the effectiveness of the treatment.
Medication:
Those diagnosed with EoE might be recommended to take a daily Proton Pump Inhibitor (PPI) medication. The function of PPIs is to reduce stomach acid. If symptoms improve with this treatment, the doctor will perform another endoscopy to see if there are still eosinophils in the esophagus. If not, the diagnosis is likely GERD (Gastroesophageal Reflux Disease) with eosinophil excess or another condition that responds to PPIs. If symptoms and eosinophils persist, the diagnosis is likely EoE.
In that case, the American College of Gastroenterology often advises using topical steroids (swallowed, not inhaled) for two months. In the United States, doctors typically recommend fluticasone or budesonide. If patients do not respond to the topical steroid, systemic steroids like Prednisone may be used. If symptoms come back, patients may need a longer period of steroids, and possibly inclusion of the ‘six food elimination diet’ or the elemental diet.
Endoscopic Treatment:
If a patient experiences food becoming lodged in the esophagus, an endoscopic procedure can help by removing the impacted food. However, doctors usually propose dilating (widening) the esophagus only after patients have been adequately treated with medication or diet changes. If even then the symptoms persist, such as difficulty swallowing, endoscopic dilation is performed. “Rings” and “strictures” (narrowing) of the esophagus can safely be widened in EoE patients. It is important to let patients know that after dilation, they might feel chest pain and there’s a minor risk of complications like esophageal rupture or bleeding.
Because EoE often co-exists with allergies, it’s usually recommended that patients with diagnosed EoE also see an allergist or immunologist.
What else can Eosinophilic Esophagitis be?
Doctors may consider the following conditions when making a diagnosis:
- Achalasia
- Celiac disease
- Crohn’s disease
- Connective tissue disease
- Drug hypersensitivity
- GERD (Gastroesophageal reflux disease)
- Graft-versus-host disease
- Hypereosinophilic disease
- Pemphigus vegetans
- Vasculitis