Learn: EoE2018-05-24T07:11:57+00:00

LEARN: Eosinophilic Esophagitis (EoE): HEALTHLINE

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Medically reviewed by Jill Seladi-Schulman, PhD on August 2, 2017 — Written by Carrie Madormo, RN, MPH

*We hope you enjoy this article. For a first hand account of what EoE is like, please visit our social media pages and Un{der}diagnosed blog where the founder, Audrey Lazzara will be sharing her story.*

Eosinophilic Esophagitis

Eosinophilic esophagitis is a condition caused when certain white blood cells, called eosinophils, are found in the esophagus. It results in pain, difficulty swallowing, and heartburn. It’s a chronic allergic and immune condition. It’s also rare, affecting about 1 in 1,000 children and 1 to 3 in 10,000 adults worldwide.

The most common symptom is difficulty swallowing or eating. This is due to the buildup of white blood cells in the tissue of the esophagus. Other symptoms can vary between children and adults.

In children, symptoms can include:

  • trouble feeding or drinking
  • weight loss
  • vomiting
  • failure to thrive

Symptoms of eosinophilic esophagitis in adults can include:

  • being unable to pass food into the stomach
  • heartburn
  • chest pain
  • abdominal pain
  • food regurgitation

In rare cases, eosinophilic esophagitis can lead to a medical emergency. Having too much food stuck in your esophagus or vomiting up food may result in a tear in your esophagus. This is rare, but needs emergency treatment right away.

This condition is mostly associated with people who have food and environmental allergies, asthma, eczema, and celiac disease.

Eosinophilic esophagitis is caused by the overabundance of eosinophils in your esophagus. This overabundance of eosinophils most likely comes from one of two causes.

Allergic response

Eosinophils in the esophagus can be caused by your body’s immune response to certain allergens. If you’re sensitive to a particular food or environmental allergen, your body could respond with eosinophilic esophagitis.

You may notice that you experience difficulty swallowing or intense heartburn after eating certain foods, such as dairy or soy. You could also be reacting to something in the environment, such as pollen.

Eosinophilic esophagitis isn’t always tied to food, but your diet is a good place to start exploring for causes.

Genetics

Another possible cause has recently been discovered. Researchers have found that many people with eosinophilic esophagitis have a higher expression of the gene eotaxin-3.

This gene helps control how many eosinophils your body makes. A higher expression of the gene means you’d produce more of the white blood cells that cause this condition.

Although this is a genetic factor, there doesn’t seem to be a strong family history component.

The only way to definitively diagnose eosinophilic esophagitis is with an endoscopy.

In this procedure, your gastrointestinal physician inserts a thin tube with a camera down your esophagus while you’re under sedation. The doctor gathers biopsies of tissue during the procedure. These are later sent off to the pathologist to test for eosinophils.

Your physician will also check your esophagus for other changes, such as:

  • inflammation or swelling
  • narrowing
  • white patches
  • creases

These signs alone aren’t enough for your doctor to diagnose eosinophilic esophagitis, but they do give your medical team a clue about what’s going on. Once your biopsy results come back from the pathologist, your physician can determine if the cause of your symptoms is eosinophilic esophagitis.

If you see an allergist for your diagnosis, they may also order blood tests to test for food allergies. The findings could help determine what foods to avoid when managing your eosinophilic esophagitis.

Your doctor will recommend a treatment based on your unique case. It may involve one or a combination of medication, natural remedies, diet changes, and surgery.

Throughout your treatment, your doctor may recommend additional endoscopies and biopsies to monitor your improvement.

Medication

The U.S. Food and Drug Administration (FDA) hasn’t approved a drug to specifically treat this condition. However, other medications can help treat its symptoms.

For example, corticosteroids can help control the inflammation in your esophagus that makes swallowing so difficult. Your physician may prescribe a higher dosage to get the swelling under control and then lower your dosage over time.

Proton pump inhibitors (PPIs) can help control the amount of acid in your stomach and esophagus. They’re used to treat acid reflux. Taking a PPI could decrease the number of eosinophils found in your esophagus and help bring down the inflammation.

Diet

Because eosinophilic esophagitis could be caused or made worse by an allergic response to certain foods, your treatment may include eliminating those foods from your diet. The challenging part is determining which foods are problematic.

This is because with this condition, food reactions often take several days to show up. It can be difficult to remember exactly which food you ate a few days ago that’s now causing an allergic response.

If you have a known food allergy based on pinprick testing, your physician will most likely start by recommending you eliminate that food right away.

If you’re not sure if you have any food allergies, start by eliminating common food allergens. These include:

  • dairy
  • eggs
  • wheat
  • soy
  • peanuts
  • tree nuts
  • fish and shellfish

In an elimination diet, all of these foods are completely removed from your diet, then slowly reintroduced one by one to determine if you’re sensitive to any of them. You may also want to try eliminating less common food allergens.

Dilation

If the above treatments aren’t helpful, your doctor may recommend a dilation procedure.

People with eosinophilic esophagitis often experience a narrowing of their esophagus, which makes eating difficult. During a dilation, your physician stretches your esophagus to make it slightly wider. This can help you swallow more easily.

However, this treatment is usually not recommended unless the other options haven’t worked.

Medical researchers are still learning about eosinophilic esophagitis and how best to treat it.

This condition is chronic and recurring without a known cure. The current treatments and medications are meant to control the buildup of eosinophils and resulting symptoms. Talk with your doctor about the best plan for you. With the right treatment, you can reduce the discomfort in your throat.

ARTICLE RESOURCES:

Q & A with Dr. Brian Riff, M.D. (Director of Endoscopy, AGMG)

To learn more about Dr. Brian Riff and his practice, please visit this link https://www.agmg.com/brianriff/

Thank you Dr. Riff! With the help of doctors like you, we can spread awareness. The donation of your time and expertise is very appreciated. You’re awesome!

*We hope you enjoy this Q & A. For a first hand account of what EoE is like, please visit our social media pages and Un{der}diagnosed blog where the founder, Audrey Lazzara will be sharing her story.*

Frequently Asked Questions about Eosinophilic Esophagitis (EoE)

How well understood is EoE by medical professionals?

Eosinophilic Esophagitis (EoE) is an emerging chronic disorder that is only recently becoming known to gastrointestinal and allergic specialists. Despite EoE being relatively common (approximately 0.4% prevalence rate among children and adults), it is still not well known among primary care physicians. EoE is as common as Crohn’s disease but is much less known by the general and medical profession. Part of the reason EoE is not as well known is that EoE was only first described in 1968. But it wasn’t until the 1990’s that EoE was recognized as a distinct disease and not until 2009 that EoE was given its own unique diagnosis code. Prior to that, esophageal eosinophilia was thought to be exclusively a complication of gastroesophageal reflux disease (GERD). Now that we know that EoE is a distinct allergic disorder, gastroenterologists are more in tune with the subtle symptoms. As such, the diagnosis has actually increased 70 fold over the last 15 years.

How is EoE diagnosed? Different well renowned institutes have varying definitions of what EoE is.

A consensus definition for EoE was not established until 2011. Prior to that, there were a variety of definitions which may be still found around the internet or used by medical providers who aren’t up to date with current treatment. In order to make the diagnosis of EoE, three specific criteria must be met. The official definition requires the presence of at least 15 eosinophils per high power field on esophageal biopsies. In layman’s terms, that means that the pathologist can count at least 15 eosinophils (a specialized white blood cell) in every field of the view under the microscope.  The eosinophils can only be seen in biopsies from the esophagus and not in the stomach and the small intestine. Lastly, the eosinophils must still be present even after acid reflux is completely treated with high dose acid suppressive medicine for 2 months.


Is EoE considered an autoimmune disease, allergic/immune disease, a swallowing disorder or none of the above? Please elaborate.

EoE is considered an allergic condition. We know this because one of the most effective treatment involves removing specific allergens from coming into contact with the esophagus. In addition, many patients with EoE also have other allergic disorders such as asthma and seasonal, food or contact allergies. Research has shown that the major driver of EoE is a cell called T-helper cell 2 (Th2). Th2 is responsible for many of the allergic disorders in humans. Patients with EoE have a distinct genetic disposition to having Th2 mediated problems which is why many patients have multiple allergic conditions. To further show that this is a genetic problem, the sibling of a patient with EoE is 80x more likely to also have EoE.  In addition, men are 3x more likely to have EoE compared to woman. If untreated, the chronic inflammation produced by activated Th2 and many other mediators eventually causes scar tissue to form in the lining of the esophagus. This scar tissue (fibrosis) affects the ability of the esophagus to contract and ultimately causes the esophagus to narrow. In good news, there is no cancer risk or reduced life expectancy with EoE.

How is EoE diagnosed?

The first part of diagnosis EoE is to get a careful history. Physicians should think about EoE in patients who report difficulty swallowing especially if there is a history of food getting stuck in the esophagus. The disease can present with a wide range of symptoms. In children, there might be poor weight gain, nausea and vomiting. In adults, patient are more likely to present with trouble swallowing, feeling like food getting stuck or with reflux/heartburn symptoms that do not respond to acid medicines. Patients unintentionally change the way they eat such as eating slowly, cutting food into small pieces or even avoiding eating certain foods without realizing it because of their disease. Alcohol is known to exacerbate the symptom of heartburn in patients with EoE. As such, physicians give many patients the diagnosis of GERD if they are not carefully listening to their symptoms.

If EoE is suspected, the diagnosis can only be made via endoscopy (a procedure where a thin flexible scope is passed into the esophagus and stomach to obtain biopsies). At least 4 separate biopsies should be performed from the top to the bottom of the esophagus since the disease may have patchy involvement.  A key part of the diagnosis is making sure there is no co-existing acid reflux. As such, patients must still have the eosinophils on biopsies even on high dose of acid suppressive medicines. Because of this it may require two endoscopies to sure the diagnosis – the initial EGD and then the second one 2 months later on high dose acid medicine.

Once diagnosed, what are the treatment options? If a patient is unresponsive to these treatments, what are their other options?

The goal of treating EoE is to improve symptoms, control inflammation and restore function of the esophagus. We know that long term active EoE can cause narrowing of the esophagus called a stricture. There are three major treatment strategies that can be used with EoE: drugs, diet or dilation.

Drugs – steroids can target key markers of inflammation and decrease scar tissue formation. The two most common steroids used are fluticasone or budesonide. Unlike in asthma, the steroids are swallowed not inhaled. Not all patients will respond to steroids. The response rate ranges from 53-95% depending on the study.  For patients who do not respond to the topical steroids, other drugs such as pill steroids can be tried. Alternatively, diet or dilation may be used. Lastly, there are many exciting medicines being researched for EoE which we hope will revolutionize therapy in the next few years. Some patients who do not respond to any current medicines may be enrolled in clinical trials to test these medicines.

Diet – dietary avoidance diets have been a mainstay therapy for EoE for many years. This was first identified when several children with EoE responded to a special liquid formula diet called elemental. This liquid diet has all potential food allergies removed. Since then, research has identified the 6 major food triggers of EoE to be wheat, milk, soy, nuts, eggs and seafood. This is called the “Six Food Elimination Diet (SFED)”. The treatment involves removing all of the offenders up front and then slowly introducing each group one by one back into the diet. Repeat endoscopy is performed with the introduction of each food item to assess how the esophagus responds. Of note, the pathway for allergic inflammation of the esophagus in EoE is different than most traditional allergies. As such, skin testing is not accurate in predicting which foods will be the trigger and allergy shots are not effective in EoE.

Dilation – Some patients can be managed with just mechanical therapy alone. Instead of anti-inflammatory medicines or eliminating the allergan in the diet, the esophagus is regularly stretched to eliminate the narrowing caused by EoE. Unfortunately, the narrowing tends to recur since the source of inflammation is not being treated.


How should the pain associated with swallowing be dealt with?

As stated before, the classic symptoms of EoE include difficulty swallowing, feeling like food is getting stuck and heartburn. Some patients may experience other symptoms such as nausea, vomiting, weight loss or pain with swallowing. The primary treatment is typically steroids or diet changes as  discussed above. Symptoms should improve with decreased inflammation in the esophagus. Patients should make sure they eat slowly, all food should be cut up and chewed well, take sips of water in between each bite. These lifestyle measures make sure that the food is well lubricated and goes down the food pipe with ease.  If symptoms persist, topical numbing medicines or antacids can help with painful swallowing and dilation can help if food is getting stuck on a narrowed portion of the esophagus


Can EoE cause abdominal pain?

Yes definitely. EoE can cause a number of symptoms. In children, the symptoms can be quite varied such as abdominal pain, vomiting, nausea or difficulty gaining weight. Symptoms in adults are typically more localized to the chest such as trouble swallowing, getting food stuck or persistent heartburn but abdominal pain can be seen. Interestingly enough, some studies suggest that patients with EoE who have abdominal pain as one of their major symptoms are less likely to respond to drug or diet therapy.


Can EoE be cured at this time?

EoE is a chronic disease and there is no cure available at this time. One of the major areas of research in EoE is how long patients should be on medicines. We know that in most patients with EoE, stopping therapy will result in recurrent symptoms. As such most patients are recommended to stay on the lowest effective medicine or diet to limit development of strictures over time.


How can the EoE community best contribute to finding a cure and helping spread awareness?

EoE is a very active area of research with a need for strong patient awareness. This is a very common disorder with effective treatments. Unfortunately many patients with EoE have modified their diet or eating habits on their own because of their swallowing difficulties. In addition, some patients think its normal for food to get stuck occasionally.  Patients should be encouraged to discuss any swallowing problem with their doctor. Over time, narrowing of the esophagus can occur and present as an emergency with food getting lodged in the esophagus. Any friend or family member should discuss their symptoms with their doctor.

Are there related diseases to EoE? Many patients with EoE also have other diseases.

There are other less common eosinophilic disorders of the GI tract such as eosinophilic gastritis (stomach), enteritis (small intestine) or colitis (colon). Collectively including EoE, these disorders are called eosinophilic gastrointestinal disorders. Symptoms can include abdominal pain, diarrhea, nausea, vomiting, weight loss and feeling full easy. Because these other disorders are very rare, treatment and prognosis are not as well as established compared to EoE.

As discussed earlier, patients with EoE typically have other allergic disorders such as asthma, allergic rhinitis (seasonal allergies) or food/skin allergies. Besides the allergic conditions, patients with EoE also have increased rates of celiac disease (gluten sensitivity), Crohn’s disease and connective tissue disorders such as Marfan syndrome or Ehlers-Danlos syndrome.

Many EoE patients report that they struggle with extreme fatigue, body aches and chronic fevers. Could this possibly be linked to EoE or is it most likely another underlying problem?

A recent study was conducted in the UK on the quality of life of patients with EoE. We know the symptoms of EoE can be uncomfortable and embarrassing in social situations (eating in public). As such it is not surprising that EoE might have an effect on the overall quality of life. Compared to patients of the same age/gender without EoE, patients with EoE had lower quality of life mental health scores including depression, anxiety and general psychological well being. In addition to lower mental health scores, EoE patients also had a lower general perception of their own health with lower energy and vitality. Not surprisingly, the quality of life scores were dependent on the severity of the EoE symptoms. The better the EoE was controlled, the better the patients felt overall.

Do you believe that EoE should be considered a disability in patients that do not respond to treatment? How should schools and workplaces accommodate people with EoE?

The definition of disability and the protections provided under the Americans with Disabilities Act is a legal question that is beyond my scope of knowledge. Per the American Partnership for Eosinophilic Disorders (APFED, apfed.org) EoE is considered a disability according to section 504 of the Rehabilitation Act of 1973. As such, patients with EoE are entitled to develop a 504 plan with their school district. In addition to protections at public schools, there are legal protections for patients with food allergies in the workplace. This is an area to discuss with your human resource personnel and obtain legal representation as needed.