Eosinophilic Oesophagitis

Eosinophilic oesophagitis is a chronic condition where a type of white blood cell called eosinophils builds up in the tube connecting your mouth to your stomach, causing inflammation that can make swallowing difficult and painful.

Table of contents

• What is eosinophilic oesophagitis?
• Associated anatomy
• Symptoms
• Causes and risk factors
• Diagnosis
• Treatment approaches
• Complications
• Living with eosinophilic oesophagitis

What is eosinophilic oesophagitis?

Eosinophilic oesophagitis, also known as EoE, is a chronic disease that affects the oesophagus, the muscular tube that carries food and liquids from your mouth to your stomach^[1]. This condition was first described only in the early 1990s, but is now recognised as a major cause of digestive system illness^[1].

In this disease, a type of white blood cell called an eosinophil builds up in the lining of the oesophagus^[1]. Normally, there are no eosinophils in the oesophagus^[5]. When eosinophils accumulate, they can release substances into surrounding tissues that cause inflammation^[5]. This buildup is a reaction to foods, allergens, or acid reflux, and can inflame or injure the oesophageal tissue^[1].

Without treatment, this inflammation can lead to scar tissue and narrowing in your oesophagus, making it difficult to swallow^[2]. When you take a bite of food, that bite cannot easily move through your oesophagus to your stomach. You may feel like something is stuck or moving slowly through your oesophagus^[2].

Eosinophilic oesophagitis is a lifelong health issue^[3]. There is no cure for it, but it can be controlled^[3]. Most people with this condition will need treatment for the rest of their lives^[2].

The condition once was rare, but the number of cases is increasing^[2]. It affects about 34 in 100,000 people^[2]. Other sources estimate it occurs in approximately 1 out of 1,700 people^[3]. Anyone, from babies to adults, can have it^[2]. However, it is more common in males and affects both adults and children^[4].

EoE, Allergic oesophagitis

Associated anatomy

• Oesophagus

Symptoms

The symptoms of eosinophilic oesophagitis vary with age. Infants and toddlers may refuse to eat or not grow properly^[5]. School-age children often have decreased appetite, recurring abdominal pain, and trouble swallowing or vomiting^[5]. Teenagers and adults can have the same symptoms, but often have difficulty swallowing dry or dense, solid foods^[5].

In adults, the main symptoms include difficulty swallowing (also called dysphagia), food getting stuck in the oesophagus after swallowing (also known as impaction), chest pain that is often centrally located and does not respond to antacids, and backflow of undigested food (known as regurgitation)^[1]. Adults may also experience heartburn or chest discomfort^[2].

In children, symptoms include difficulty feeding in infants, difficulty swallowing, vomiting, poor weight gain and growth, and reflux that does not get better with medicines^[6].

Symptoms that are less common but may affect people of any age include abdominal (belly) pain, nausea and vomiting, and regurgitation^[2]. Food impaction, where food gets stuck in your oesophagus, may be a medical emergency and require a trip to the emergency room because it can tear a hole in your oesophagus^[2].

The difficulty swallowing occurs because the oesophagus is inflamed, and in severe cases, because the oesophagus narrows to the point that food gets stuck^[5]. Due to long-standing inflammation and possible resultant scarring, adults presenting with eosinophilic oesophagitis tend to have more episodes of oesophageal food impaction as well as other oesophageal abnormalities^[4].

In some cases, people have symptoms that come and go. A healthcare practitioner may call this a flare-up^[2]. Symptoms may feel like symptoms of more common oesophageal disorders. People with this condition may have it for several years before they receive a correct diagnosis^[2].

Causes and risk factors

The disease happens when your immune system reacts to allergens in your oesophagus, filling it with inflammatory cells, including eosinophils and cytokines^[2]. Allergens can be environmental or food triggers^[2].

While the exact cause is not known, the general belief is that it is typically caused by an immune response to specific foods^[3]. The pathophysiology is incompletely understood, but it is thought to involve some type of antigen exposure, coupled with a pre-existing genetic susceptibility, which causes a hyperactive immune response from immune cells in the oesophagus^[4].

The most common food triggers are dairy, egg, peanuts and tree nuts, seafood and shellfish, soy, and wheat^[2].

Many people with eosinophilic oesophagitis also have asthma, allergic rhinitis, atopic dermatitis, eczema, and food allergies^[2]. People with this condition commonly have other allergic diseases such as rhinitis, asthma, or eczema^[3].

Another risk factor is having a family history of eosinophilic oesophagitis^[2]. Certain families may have an inherited tendency to develop this condition^[3]. Researchers have identified a number of genes that play a role in eosinophilic oesophagitis^[7].

Diagnosis

To diagnose eosinophilic oesophagitis, your healthcare provider will consider both your symptoms and test results^[8]. This will include determining whether you have gastroesophageal reflux disease (GERD)^[8].

Currently, performing an upper endoscopy with biopsies of the oesophagus is necessary to diagnose eosinophilic oesophagitis^[5]. An endoscopy is a medical procedure where a flexible tube containing a light source and a camera lens is passed down the oesophagus so your doctor can see if your oesophagus is inflamed^[5].

During an endoscopy, your provider will use a long, narrow tube (endoscope) containing a light and tiny camera and insert it through your mouth down the oesophagus^[8]. The lining of your oesophagus will be inspected for inflammation and swelling, horizontal rings, vertical furrows, narrowing (strictures), and white spots^[8]. Some people with eosinophilic oesophagitis will have an oesophagus that looks typical^[8].

A biopsy will be done during the endoscopy. A biopsy involves taking a small bit of tissue. Multiple tissue samples will likely be taken from your oesophagus and then examined under a microscope for eosinophils^[8]. Small pieces of oesophageal tissue the size of the tip of a pen (biopsies) are taken to be examined under the microscope^[5]. The samples will be checked for a high number of eosinophils. This is the only way to make a diagnosis^[6].

You may be given blood tests to look for higher than usual eosinophil counts or total immunoglobulin E levels, suggesting an allergy^[8]. If you do have eosinophilic oesophagitis, you may have blood or other types of tests to check for specific allergies^[6].

Eosinophils can be found in the oesophageal tissue in diseases other than eosinophilic oesophagitis. One common example is acid reflux disease. Other diseases with eosinophils in the oesophagus must be ruled out before this condition can be accurately diagnosed^[5].

Treatment approaches

There is no cure for eosinophilic oesophagitis. Treatments can manage your symptoms and prevent further damage^[6]. The currently accepted therapies are categorised as diet, drugs, and dilation^[12]. The first line of treatment in a patient with uncomplicated disease can be either a dietary or a drug-based approach^[12]. Choosing which approach to pursue is typically based on shared decision-making between the patient, their caregivers (or parents in paediatric patients), and their healthcare providers^[12].

Dietary changes

Dietary changes can be effective in resolving inflammation, although studies are limited by lack of control groups and short durations^[13]. An elemental diet, consisting of amino acid-based formulas, resolves inflammation in more than 90% of children, with less benefit in adults^[15]. However, most patients will not maintain an elemental diet because of taste, nutritional concerns, and implementation barriers^[15].

An empiric six-food elimination diet resolves inflammation in two-thirds of patients^[15]. This involves stopping eating and drinking certain foods and beverages for several weeks. If you are feeling better, you add the foods back to your diet one at a time. You have repeat endoscopies to see whether or not you are tolerating those foods^[6].

With one type of elimination diet, you first have an allergy test. Then you stop eating and drinking the foods you are allergic to^[6]. Eliminating foods based on allergy testing results resolves inflammation in one-half of patients^[15].

Recent research has found that forgoing one food, specifically animal milk, treats eosinophilic oesophagitis as well as excluding six foods^[21]. Eliminating animal milk alone from the diet of adults is as effective at treating the disease as eliminating animal milk plus five other common foods^[21]. For people whose disease remains active after they forgo animal milk, a more restrictive diet may help them achieve remission^[21].

Medicines

The two main types of treatments are medicines and diet^[6]. The primary treatments include proton pump inhibitors and topical steroids^[15].

Steroids can help control inflammation. These are usually topical steroids, which you swallow either from an inhaler or as a liquid^[6]. Topical steroids are more effective than proton pump inhibitors, resolving inflammation in two-thirds of patients^[15]. Sometimes doctors prescribe oral steroids (pills) to treat people who have serious swallowing problems or weight loss^[6].

Acid suppressors such as proton pump inhibitors (PPIs) may help with reflux symptoms and decrease inflammation^[6]. Proton pump inhibitor use resolves inflammation in only one-third of patients, but this is more than double the response to placebo^[15].

Monoclonal antibodies can reduce inflammation and may help with swallowing. They can be used in adults and children over age 12^[6].

Endoscopic dilation

Endoscopic interventions, such as oesophageal dilation, are generally considered for patients with complicated eosinophilic oesophagitis^[12]. Dysphagia in this condition is primarily caused by strictures secondary to inflammation^[15]. Endoscopic dilation leads to symptom improvement in 87% of patients^[15]. In severe cases, it may be necessary to enlarge the oesophagus with an endoscopy procedure^[4].

Complications

Damage to your oesophagus from eosinophilic oesophagitis may cause the following complications: oesophageal perforation (rupture) and oesophageal strictures^[2].

Left untreated, eosinophilic oesophagitis progresses to fibrous remodelling and stricture formation that impairs quality of life^[13]. A delay in diagnosis or uncontrolled eosinophilic inflammation in the oesophageal epithelium can be associated with suboptimal quality of life^[12].

The condition does not appear to limit your life and there is currently no strong data suggesting it causes cancer of the oesophagus^[3].

Living with eosinophilic oesophagitis

Eosinophilic oesophagitis is considered to be a chronic condition that can be treated but is not outgrown^[5]. It is a lifelong disease that requires either repeated treatments or maintenance therapy^[13].

Working with your healthcare team, including a gastroenterologist, nutritionist, and allergist, you can make a health and nutrition plan just for you to help put you in control of your symptoms and your life^[3].

Eating is a big part of social occasions, and the uncertainty and embarrassment of symptoms can really weigh on you^[18]. People do a lot of creative things to adapt to life with this condition. Over time, people get accustomed to these coping methods and forget how much they have to adapt. But these workarounds are only temporary solutions that do not address the underlying problem of chronic inflammation^[18].

The biggest problem with workarounds is that you get used to them. This may delay you from getting properly diagnosed^[18]. If you are relying on workarounds such as drinking a lot of fluids with meals to wash food down, avoiding certain foods, using a lot of sauce, chewing food excessively, eating very slowly, or cutting food into tiny pieces, let your doctor know, because these are not a long-term solution^[18].

If you are getting overwhelmed by your diagnosis, do not wait to reach out to a mental health expert to talk through the changes^[3]. There are a number of support groups for caregivers and patients. Reach out to your healthcare provider, registered dietitian, local hospital, or community centre for more details^[3].