Eosinophilic Esophagitis: Introduction and Implications for Feeding

Editor's Note: This text is a transcript of the webinar, Eosinophilic Esophagitis: Introduction and Implications for Feeding, presented by Rhonda Mattingly, Ed.D, CCC-SLP.

Introduction and Overview

This is the final course in our five-part series on medical conditions that underlie pediatric feeding disorders. If you happened to miss the first four sessions, they can all be found on this SpeechPathology.com landing page:  

https://www.speechpathology.com/pediatric-feeding-series/

Today, we're going to discuss eosinophilic esophagitis (EoE). Interestingly, this condition has not been recognized for that long, in comparison to many of the more familiar diagnoses that are frequently associated with feeding difficulties. In the 1970s, there were cases suggestive of EoE, but it wasn't until the 1990s that a physician distinctively identified the disorder and named it EoE (Kelly et al., 1995). Many of the feeding issues that arise in conjunction with EoE, you will also find with other disorders. Much of this presentation is to provide an overview of this condition, but we will also spend some time discussing the role of speech-language pathologists in assisting with these cases. Additionally, we will discuss several strategies for helping treat and manage individuals with this disorder. 

EoE Defined

Eosinophils are a type of disease-fighting white blood cell. Their function is to protect us, to fend off bacteria and parasites and to help kill cells that need to be killed. However, high amounts of eosinophils can cause autoimmune conditions, which develop when the body's immune system decides that healthy cells are foreign and begins to attack them.

Typically, eosinophils exist in the gastrointestinal region, such as in the colon, the duodenum, and the gastric antrum. The esophagus, however, typically does not have eosinophils. It is possible get some eosinophils from reflux, but that's a different issue that we will discuss shortly. Suffice it to say, eosinophils are not supposed to be in the esophagus, and certainly not in the quantity that they are in individuals with EoE.

Diagnostic Guidelines and Consensus Report

In the early 1990s, EoE was defined as a distinct clinicopathological diagnosis. In other words, it cannot be diagnosed simply from a pathology study or from a clinical study: you have to do both. In 2007, a number of gastroenterologists with experience in EoE collaborated and authored a position statement on how to diagnose EoE. They outlined the clinical symptoms of EoE, which include vomiting, abdominal pain, heartburn, dysphagia, reflux symptoms, and avoidance of feeding and eating. In addition, they agreed that the patient must have multiple biopsies of different areas of the esophagus, because it can be worse in certain areas than in others. A positive result for EoE would be greater than 15 eosinophils per 40x high-power field. They also determined that they would evaluate the histology of all other GI Tracts to be normal, in order to exclude any other GI diagnoses, as clinically, some of these diagnoses (e.g., GERD, Crohn's disease, etc.) are similar to EoE. 

In the mid- to late-2000s, there had been a dramatic increase in the amount of research being conducted in the area of EoE. In response to this research, a task force of GIs assembled in 2011 and created a consensus report on EoE. This team consisted of 33 GIs with experience working with pediatric and adult patients. Based on the new research findings, their intent was to modify some recommendations related to the diagnosis of EoE, as well as expand on some key points. They arrived at the following definition of EoE: "Eosinophilic esophagitis represents a chronic, immune/antigen mediated, esophageal disease characterized clinically by symptoms related to esophageal dysfunction and histologically by eosinophil-predominant inflammation.”

To reiterate, the diagnostic guidelines require us to assess the person in two ways: clinically and pathologically. From a clinical standpoint, we need to determine whether they have the esophageal dysfunction. Furthermore, we also have to ascertain pathologically if they have one or more biopsies revealing 15 or more eosinophils in a peak high-powered field, with those biopsies being taken from the esophagus in several different locations in the esophagus.

Proton Pump Inhibitors (PPI)

In order to properly diagnose EoE, other causes for the presence of eosinophils must be ruled out. One of the ways they do that is by using a trial of PPIs (proton pump inhibitors). PPIs are a group of pharmacological agents whose main purpose is to block the production of stomach acid. An individual will be prescribed a PPI for several weeks, and then evaluated. If the person's condition improves, EoE can be ruled out. However, if there's no response to that PPI, that is an indication that the individual likely has EoE. The thinking is that the EoE is what's causing all of those eosinophils to be in the esophagus.

If PPIs are effective, that raises the question of whether there is a condition known as PPI responsive esophageal eosinophilia (PPI-REE). Without going into too much detail, PPI responsive esophageal eosinophilia is a disease that has the same features of EoE, but is responsive to PPIs. Some controversy exists about the status of the diagnosis of PPI-REE. One possible explanation is that patients with PPI-REE have GERD-induced esophageal eosinophilia that responds to PPIs, though pH and endoscopic monitoring has been normal. The second thought is perhaps there are certain anti-inflammatory effects of PPIs that we're not aware of that are impacting some aspect of EoE. Suffice to say, it's not an agreed upon condition or term.

Associated Diagnoses

There are other diagnoses that are associated with EoE, because they have similar clinical symptoms, which may cause some confusion. Before an official EoE diagnosis can be made, and prior to performing any biopsies, these other conditions must first be ruled out. As SLPs who treat children with feeding problems, it's important for us to be aware of these conditions.

• Crohn's Disease: This is an inflammatory bowel disease characterized by severe/chronic inflammation of the intestinal wall or a portion of the GI tract. The ileum and the rectum are most commonly affected; however, the esophagus may be involved. Symptoms include diarrhea, abdominal fever and weight loss. The cause of Crohn's Disease is unknown. Some of the clinical symptoms of Crohn's Disease which may impact feeding are similar to EoE symptoms. 
• Eosinophilic Gastroenteritis: This is a condition that shares many common features of EoE, however, it has a lower incidence than EoE, and it involves a different site of disease. Some clinical manifestations include nausea and vomiting, or bowel obstruction. Systemic corticosteroids can be used to treat it, but if they are used for long periods of time, it may result in corticosteroid toxicity. 
• Hypereosinophilic Syndrome: This is a rare blood disorder, where the blood contains high number of eosinophils. A normal level of eosinophils in the blood is about 500 per microliter; an individual with hypereosinophilic syndrome will have 1500 eosinophils per microliter. This high level of eosinophils will begin to cause inflammation in varying tissues, eventually resulting in organ dysfunction. The most commonly involved organs are skin, lungs, heart and nervous system. Symptoms include rash, dizziness, memory loss and confusion, cough, shortness of breath, fatigue, fever and mouth sores.
• Celiac Disease: This is an inherited autoimmune disorder which affects the digestive process of the small intestine. Celiac disease is typically found in the duodenum and the jejunum. In those with Celiac disease, the immune system reacts to the intake of gluten by attacking the small intestine. The result is malabsorption of nutrients. A person may be asymptomatic, or it may result in the following symptoms: abdominal bloating, chronic diarrhea or constipation, vomiting, liver/biliary tract disorders, weight loss, pale stool, iron-deficiency anemia, fatigue, FTT, delayed puberty, and/or joint pain.
• Achalasia: With Achalasia, nerve cells in the esophagus degenerate due to an unknown cause. As a result of the degeneration, the esophageal muscles do not contract normally, and the lower esophageal sphincter does not function properly. The most common symptom of Achalasia is difficulty swallowing. Other symptoms include chest pain, difficulty burping, heartburn, regurgitation, the sensation of food being "stuck", hiccups and/or weight loss.

Eosinophilic Esophagitis

Incidence and Prevalence

The prevalence of EoE is increasing, which may be due in part to increased awareness and knowledge of the disorder. As of 2004, there were one to two cases in 10,000 documented (Noel et al., 2004). In 2012, four to five cases in 10,000 were documented (Liacouras et al., 2012). It is three times more prevalent in males than females (Furuta et al., 2007). EoE is a chronic condition: there are ways to manage it, but not to cure it. In addition, there have been multiple reports of familial clustering with EoE (i.e., the occurrence of EoE within some families is greater than what would be expected from the occurrence in the general population).