Q & A with Dr. Brian Riff, M.D.

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.