Eosinophilic Esophagitis

  • Eosinophilic esophagitis is an inflammatory condition of the esophagus that affects both children and adults, and men more than women.
  • Eosinophilic gastroenteritis may be a due allergy to an as yet unknown food allergen.
  • The primary symptom in adults with eosinophilic esophagitis is dysphagia (problems swallowing) for solid food.
  • Eosinophilic esophagitis stiffens the esophagus so that solid foods have difficulty passing through the esophagus and stomach. Eosinophil deposition in the esophagus can be in the form of ridges or rings, impeding the passage of food.
  • Other common causes of dysphagia for solid food are esophageal strictures and Schatzki rings.
  • The diagnosis of eosinophilic esophagitis usually is made during an endoscopy (EGD) and performed to evaluate dysphagia. The diagnosis is confirmed by biopsy of the esophagus.
  • The treatment of eosinophilic esophagitis is with proton pump inhibitors and swallowed fluticasone propionate or budesonide.
  • Gentle esophageal dilatation is used when medications fail to relieve dysphagia.

Eosinophilic Esophagitis

Eosinophilic esophagitis is an inflammatory condition in which the esophagus wall becomes filled with large numbers of eosinophils, a type of white blood cell.

Causes of Eosinophilic Esophagitis

The esophagus is a muscular tube that propels swallowed food from the mouth into the stomach. Esophagitis refers to inflammation of the esophagus that has several causes.

  • The most common cause of esophagitis is acid reflux, which most frequently results in heartburn. Acid reflux can also cause ulcers in the inner lining of the esophagus.
  • Other less common causes of esophagitis include viruses (such as herpes simplex), fungi (such as Candida), medications that become stuck in the esophagus (such as the antibiotic, tetracycline), and radiation therapy (such as during treatment of lung cancer).

Doctors believe that eosinophilic esophagitis is a type of esophagitis caused by an allergen for two reasons.

  • First, eosinophils are prominent in other diseases associated with allergies such as asthma, hay fever, allergic rhinitis, and atopic dermatitis.
  • Second, people with eosinophilic esophagitis are more likely to suffer from these other allergic diseases.

Nevertheless, the exact substance that causes the allergic reaction in eosinophilic esophagitis is not known. The hallmark of eosinophilic esophagitis is large numbers of eosinophils in the tissue just beneath the inner lining of the esophagus.

Eosinophils are white blood cells (leukocytes) manufactured in the bone marrow. They are one of the many types of cells that actively promote inflammation. They are particularly active in the kind of inflammation caused by allergic reactions. Thus, many eosinophils can accumulate in tissues such as the esophagus, the stomach, the small intestine, and sometimes in the blood when individuals are exposed to an allergen. As previously mentioned, the allergen(s) that causes eosinophilic esophagitis is not known. It is not even known whether the allergen is inhaled or ingested.

Eosinophilic esophagitis affects both children and adults. For unknown reasons, men are more commonly affected than women, and it is most commonly found among young boys and men.

Symptoms Eosinophilic Esophagitis

The primary symptom in adults with eosinophilic esophagitis is difficulty in swallowing solid food (dysphagia). Specifically, the food becomes stuck in the esophagus after it is swallowed.

Less common symptoms include heartburn and chest pain.

In children, the most common symptoms are

  • abdominal pain,
  • nausea, vomiting,
  • coughing, and
  • failure to thrive.

Eosinophilic Esophagitis causes Dysphagia

Eosinophilic esophagitis decreases the esophagus’s ability to stretch and accommodate mouthfuls of swallowed food, probably due to the presence of so many eosinophils and, perhaps, due to some scarring that occurs in the wall of the esophagus. As a result, solid foods (particularly solid meats) have difficulty passing through the esophagus. When solid food sticks in the esophagus, it causes an uncomfortable sensation in the chest. The sticking of food in the esophagus is referred to as dysphagia. If the solid food then passes into the stomach, the discomfort subsides, and the individual can resume eating. Suppose the solid food does not pass into the stomach. In that case, individuals often must regurgitate the food by inducing vomiting before they can resume eating. Rarely, solid food becomes impacted; that is, it can neither pass into the stomach nor be regurgitated. The impacted solid food causes chest pain that can mimic a heart attack and causes repeated spitting up of saliva that cannot be swallowed because of the esophagus’s obstruction. Individuals with impacted food are unable to eat or drink. To relieve the obstruction, a doctor usually will have to insert a flexible endoscope through the mouth and into the esophagus to remove the impacted food.

How eosinophilic esophagitis causes abdominal pain symptoms, vomiting, and failure to thrive in children is not clear.

Causes of Dysphagia

The most common causes of dysphagia for solid food are esophageal strictures and Schatzki (lower esophageal) rings. Esophageal strictures are narrowings of the esophagus that result from inflammation and scarring, most commonly from chronic acid reflux. Strictures usually are located in the lower esophagus near the esophagus entrance into the stomach where the acid reflux is most severe. Schatzki rings are thin webs of tissue of unclear cause that can narrow the lumen (center) of the esophagus through which food passes. They also are located in the lower esophagus.

A less common cause of dysphagia for solid food is esophageal cancer that narrows the esophageal lumen. A still less common cause of dysphagia is disorders of the muscles of the esophagus. For example, achalasia, a disease of the nerves and the esophagus muscles, prevent the muscle at the lower end of the esophagus (the lower esophageal sphincter) from relaxing and swallowed food to pass into the stomach. Unlike the other causes of motility disorders, achalasia usually results in eating solid and liquid nutrition.

Diagnosing Eosinophilic Esophagitis

The diagnosis of eosinophilic esophagitis is suspected whenever dysphagia for solid food occurs, even though it is not one of the most common causes of dysphagia. Dysphagia is almost always evaluated by endoscopy (esophagogastroduodenoscopy or EGD) to determine its cause. A flexible viewing tube or endoscope is inserted through the mouth and into the esophagus during the EGD. This allows the doctor to see the esophagus’s inner lining (as well as the stomach and duodenum). Cancers, esophageal strictures, Schatzki rings, and usually achalasia can be diagnosed visually at the time of EGD.

The doctor performing the EGD also may see abnormalities that suggest eosinophilic esophagitis. For example, some patients with eosinophilic esophagitis have narrowing of most of the esophagus. Others have a series of rings along the entire length of the esophagus. Still, others have furrows running up and down the esophagus. A few have small white spots on the esophageal lining, which represent pus made up of eosinophils’ dying mounds. The diagnosis of eosinophilic esophagitis is established with a biopsy of the inner lining of the esophagus. The biopsy is performed by inserting a long thin biopsy forceps through a channel in the endoscope that pinches off a small sample of tissue from the esophagus’s inner lining. A pathologist then can examine the biopsied tissue under the microscope to look for eosinophils.

However, in many patients with eosinophilic esophagitis, the esophagus looks normal or will show only minor abnormalities. Unless biopsies are taken of a normal-appearing esophagus, the diagnosis of eosinophilic esophagitis can be missed. In fact, not taking biopsies has resulted in some patients having dysphagia for years before the diagnosis of eosinophilic esophagitis is made. Doctors are now more likely to perform biopsies of the esophagus in individuals with dysphagia, even those with a normal-appearing esophagus, who have no apparent cause for their dysphagia.

The incidence of eosinophilic esophagitis is on the rise in the U. S. This rise in incidence may reflect either increased awareness of the disease among the doctors treating patients with dysphagia or an actual increase in the prevalence of this disease.

Treating Eosinophilic Esophagitis

Esophageal dilatation

The treatment of eosinophilic esophagitis is with gentle esophageal dilatation and medications. The goal of treatment is to relieve symptoms of dysphagia.

For decades, gastroenterologists have been treating patients from eosinophilic esophagitis in the same manner as patients with dysphagia due to esophageal strictures and Schatzki rings. Esophageal dilatation involves physically stretching the strictures or fracturing of the strictures or fracturing the rings, thus allowing freer passage of solid food. Stretching or fracturing of the strictures or rings can be performed with endoscopes, long and flexible dilators of different diameters inserted through the mouth, or balloons inserted into the esophagus through a channel in the endoscope. The balloons are positioned at the stricture or ring level and then inflated to break the stricture or ring.

While esophageal dilatation has been a significant and usually safe treatment, doctors have observed that some patients with eosinophilic esophagitis develop tears in the esophageal lining that can lead to severe chest pain after dilation. Rare cases of esophageal perforations (tears through the entire esophageal wall) also have been reported. Esophageal perforations are a severe complication that can lead to infections in the chest. Thus, although doctors may still use dilatation to treat dysphagia from eosinophilic esophagitis, they are now more likely to use smaller dilators and less force than when treating esophageal strictures and rings. Moreover, doctors are more commonly using medications to treat dysphagia from eosinophilic esophagitis and using dilation only when drugs fail.

Medications

The medications primarily used in treating eosinophilic esophagitis are fluticasone propionate (Flovent), budesonide suspension, and proton pump inhibitors.

Fluticasone propionate (Flovent)

Although oral steroids effectively treat eosinophilic esophagitis, the side effects of orally-administered steroids limit their use. One new oral steroid being tested is budesonide. This orally-administered steroid is absorbed into the body but is rapidly destroyed, resulting in fewer serious side effects. The current treatment of eosinophilic esophagitis is with swallowed (not inhaled) fluticasone propionate. Fluticasone propionate is a synthetic (human-made) steroid related to the naturally occurring steroid hormone, cortisol or hydrocortisone, produced by the adrenal glands. These steroids have potent anti-inflammatory actions. When used as an inhaler, fluticasone propionate reduces inflammation in the airways of patients with asthma, thus relieving wheezing and breathing difficulties. When fluticasone propionate is swallowed, it has been shown to reduce the eosinophils in the esophagus and relieve dysphagia in patients with eosinophilic esophagitis.

In treating eosinophilic esophagitis, fluticasone propionate is administered with the same inhaler as for asthma without the usual spacer in the inhaler. The spacer’s removal causes the fluticasone propionate to deposit in the mouth rather than enter the lungs. The fluticasone propionate that deposits in the mouth is then swallowed with a small amount of water, usually twice daily for several weeks. Patients are instructed not to eat or drink for two hours after each treatment. Improvement in dysphagia usually is prompt within days or weeks. Most patients develop recurrent symptoms after stopping treatment require treatment and or continuous retreatment.

When used in low doses, little of the fluticasone propionate is absorbed into the body. Therefore, the side effects are minimal. One possible side effect is thrush (infection of the mouth and throat by a fungus, Candida), which is relatively easy to treat. When higher doses are used for a prolonged period, enough fluticasone propionate may be absorbed to cause side effects throughout the body. Side effects of high doses of fluticasone propionate are similar to oral steroids such as prednisone and cortisone.

Proton pump inhibitors (PPIs)

Proton pump inhibitors, pantoprazole (Protonix), esomeprazole (Nexium), rabeprazole (Aciphex), lansoprazole (Prevacid), dexlansoprazole (Dexilant), and omeprazole (for example, Prilosec, Zegerid) reduce the production of acid by the stomach. They are a very safe and effective treatment for the symptoms of acid reflux and esophagitis. Since acid reflux may aggravate esophagitis in some patients with eosinophilic esophagitis, doctors frequently use proton pump inhibitors for treating eosinophilic esophagitis. Proton pump inhibitors do not treat the underlying eosinophilic esophagitis; however, treatment with fluticasone or another steroid is usually required.