Eosinophilic esophagitis

Eosinophilic esophagitis (e-o-sin-o-FILL-ik uh-sof-uh-JIE-tis) is a chronic immune system disease. With this disease, a type of white blood cell, called an eosinophil, builds up in the lining of the tube that connects your mouth to your stomach. This tube is also called the esophagus. This buildup, which is a reaction to foods, allergens or acid reflux, can inflame or injure the esophageal tissue. Damaged esophageal tissue can lead to difficulty swallowing or cause food to get stuck when you swallow.

Eosinophilic esophagitis has been identified only since the early '90s, but is now considered a major cause of digestive system illness. Research is ongoing and will likely lead to revisions in the diagnosis and treatment of eosinophilic esophagitis.

Signs and symptoms include:

Adults:

• Difficulty swallowing, also called dysphagia
• Food getting stuck in the esophagus after swallowing, also known as impaction
• Chest pain that is often centrally located and does not respond to antacids
• Backflow of undigested food, known as regurgitation

Children:

• Difficulty feeding, in infants
• Difficulty eating, in children
• Vomiting
• Abdominal pain
• Difficulty swallowing, also called dysphagia
• Food getting stuck in the esophagus after swallowing, also known as impaction
• No response to GERD medication
• Failure to thrive, including poor growth, malnutrition and weight loss

When to see a doctor

Seek immediate medical attention if you experience chest pain, especially if you also have shortness of breath or jaw or arm pain. These may be symptoms of a heart attack.

Make an appointment with your health care provider if you experience severe or frequent eosinophilic esophagitis symptoms. If you take nonprescription medicines for heartburn more than twice a week, see your health care provider.

Eosinophils are a typical type of white blood cells present in your digestive tract. However, in eosinophilic esophagitis, you have an allergic reaction to an outside substance. The reaction may occur as follows:

• Reaction of the esophagus. The lining of your esophagus reacts to allergens, such as food or pollen.
• Multiplication of eosinophils. The eosinophils multiply in your esophagus and produce a protein that causes inflammation.
• Damage to the esophagus. Inflammation can lead to scarring, narrowing and formation of excessive fibrous tissue in the lining of your esophagus.
• Dysphagia and impaction. You may have difficulty swallowing, called dysphagia. Or food may become stuck when you swallow. This is known as impaction.
• Additional symptoms. You may have other symptoms, such as chest pain or stomach pain.

There has been a significant increase in numbers of people diagnosed with eosinophilic esophagitis in the past decade. At first, researchers thought this was due to an increase in awareness among health care providers and greater availability of tests. However, studies now suggest that the disease is becoming increasingly common, parallel to the increase in asthma and allergies.

The following risk factors are associated with eosinophilic esophagitis:

• Climate. People who live in a cold or dry climate are more likely than those in other climates to be diagnosed with eosinophilic esophagitis.
• Season. You're more likely to be diagnosed between the spring and fall, probably because levels of pollen and other allergens are higher and people are more likely to be outdoors.
• Sex. Eosinophilic esophagitis is more common in males than in females.
• Family history. Researchers think that eosinophilic esophagitis may have a genetic component because the condition sometimes runs in families. If your family members have eosinophilic esophagitis, you have a greater chance of being diagnosed.
• Allergies and asthma. If you have food or environmental allergies, asthma, atopic dermatitis, or a chronic respiratory disease, you're more likely to be diagnosed with eosinophilic esophagitis.
• Age. Originally, eosinophilic esophagitis was thought to be a childhood disease, but now it is known to be common in adults as well. The symptoms differ somewhat between children and adults.

In some people, eosinophilic esophagitis can lead to the following:

• Scarring and narrowing of the esophagus. This makes it difficult to swallow and more likely that you will have food get stuck.
• Damage to the esophagus. Because of inflammation of the esophagus, endoscopy can cause perforation or tears in the tissue that lines the esophagus. Tearing also can occur in connection with retching that some people experience when they get food stuck in the esophagus.

Your health care provider will consider both your symptoms and test results to diagnose eosinophilic esophagitis. This will include determining whether you have gastroesophageal reflux disease (GERD).

Tests to diagnose eosinophilic esophagitis include:

• Upper endoscopy. Your provider will use a long, narrow tube (endoscope) containing a light and tiny camera and insert it through your mouth down the esophagus. The lining of your esophagus will be inspected for inflammation and swelling, horizontal rings, vertical furrows, narrowing (strictures), and white spots. Some people with eosinophilic esophagitis will have an esophagus that looks typical.
• Biopsy. During an endoscopy, a biopsy of your esophagus will be done. A biopsy involves taking a small bit of tissue. Multiple tissue samples will likely be taken from your esophagus and then examined under a microscope for eosinophils.
• Blood tests. If eosinophilic esophagitis is suspected, you may undergo some additional tests to confirm the diagnosis. These tests look for the sources of your allergic reaction, also called allergens. You may be given blood tests to look for higher than usual eosinophil counts or total immunoglobulin E levels, suggesting an allergy.
• Esophageal sponge. This test is performed in the health care provider's office. It involves swallowing a capsule attached to a string. The capsule will dissolve in your stomach and release a sponge that the provider will pull out of your mouth with the string. As the sponge is pulled out, it will sample the esophageal tissues. This allows your provider to determine the degree of inflammation in your esophagus without an endoscopy.

Eosinophilic esophagitis is considered a chronic relapsing disease, meaning that most people will require ongoing treatment to control their symptoms. Treatment will involve one or more of the following:

Dietary therapy

Depending on your response to tests for food allergies, your health care provider may recommend that you stop eating certain foods. Cutting out some foods, such as dairy or wheat products, may help to relieve symptoms and reduce inflammation. Sometimes, it may be recommended to limit your diet even more.

Medication

• Proton pump inhibitor (PPI). Your provider will likely first prescribe an acid blocker such as a PPI. This treatment is the easiest to use, but most people's symptoms don't improve.
• Topical steroid. If you do not respond to the PPI, your provider will then likely prescribe a steroid, such as fluticasone or budesonide. This steroid is in a liquid form that is swallowed to treat eosinophilic esophagitis. This type of steroid is not absorbed into the bloodstream, so you are unlikely to have the typical side effects often associated with steroids.
• Monoclonal antibodies. The Food and Drug Administration (FDA) recently approved dupilumab (Dupixent) for treatment of adults and children 12 years and older with eosinophilic esophagitis. Dupilumab is a type of medicine known as a monoclonal antibody. It works to block the action of certain proteins in the body that cause inflammation. Dupilumab is given weekly via injection.

Dilation

If you experience severe narrowing, known as a stricture, of your esophagus, your provider may recommend dilation. Dilation, also called stretching, can help make swallowing easier. Dilation may be used if steroids are not helpful. Or dilation may be a choice to avoid ongoing use of medication.

If you often have heartburn, these lifestyle changes may help reduce the frequency or severity of symptoms:

• Maintain a healthy weight. Excess pounds put pressure on your belly, pushing up your stomach and causing acid to back up into your esophagus. If your weight is at a healthy level, work to maintain it. If you are overweight or obese, work to slowly lose weight — no more than 1 or 2 pounds (0.5 to 1 kilogram) a week. Ask your provider for help in creating a weight-loss strategy that will work for you.
• Avoid foods and drinks that trigger heartburn. Common triggers, such as fatty or fried foods, tomato sauce, alcohol, chocolate, mint, garlic, onion, and caffeine, may make heartburn worse. Avoid foods that you know will trigger your heartburn.
• Elevate the head of your bed. If you regularly experience heartburn at night or while trying to sleep, put gravity to work for you. Place wood or cement blocks under the feet of your bed so that the head end is raised by 6 to 9 inches (152 to 228 millimeters). If it's not possible to elevate your bed, insert a wedge between your mattress and box spring to elevate your body from the waist up.

If you think you have eosinophilic esophagitis, you're likely to start by seeing your regular health care provider. Your provider may recommend that you see a specialist in treating digestive diseases (gastroenterologist) or an allergist.

Because appointments can be brief, and because there's often a lot of ground to cover, it's a good idea to be well prepared. Here's some information to help you get ready, and what to expect.

What you can do

• Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
• Bring test results. If you are seeing a new specialist after you've had an endoscopy from another provider, bring the results with you.
• Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
• Write down key personal information, including any major stresses or recent life changes.
• Make a list of all medications, vitamins or supplements that you're taking.
• Consider taking a family member or friend along. Sometimes it can be difficult to absorb all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
• Write down questions to ask your provider.

Your appointment time is limited, so preparing a list of questions can help you make the most of it. For eosinophilic esophagitis, some basic questions to ask include:

• What is likely causing my symptoms?
• What kinds of tests do I need?
• Do I need an endoscopy?
• Is my condition likely temporary or chronic?
• What is the best course of action?
• What are the alternatives to the primary approach that you're suggesting?
• I have other health conditions. How can I best manage them together?
• Are there any restrictions I need to follow?
• Should I see a specialist? What will it cost?
• Is there a generic alternative to the medicine you're prescribing for me?
• Are there brochures or other printed material I can take with me? What websites do you recommend?
• Should I schedule a follow-up visit?

In addition to the questions you've prepared, don't hesitate to ask other questions during your appointment.

What to expect from your doctor

Your provider is likely to ask you a number of questions. Being ready to answer them may allow more time later to cover points you want to address.

• What are your symptoms?
• When did you first notice them?
• Have they been continuous or occasional?
• How severe are your symptoms?
• What, if anything, seems to improve your symptoms?
• What, if anything, appears to worsen your symptoms?
• Do your symptoms wake you up at night?
• Are your symptoms worse after meals or after lying down?
• Do you have difficulty swallowing?
• Have you ever had food get stuck while you are swallowing?
• Does food or sour material ever come up in the back of your throat?
• Do you have chest pain or stomach pain?
• Have you had an esophageal dilation?
• Have you been treated with a topical steroid or food elimination diet?
• Have you gained or lost weight?
• Do you experience nausea or vomiting?
• Are your symptoms worse at certain times of the year?
• Do you have asthma or any chronic respiratory disease?
• Do you have any allergies to foods or to anything in the environment, such as pollen?
• Does anyone in your family have allergies?
• Have you tried taking antacid or anti-reflux medication? What was the result?

If you're a parent of a young child, the provider also may ask if your child has trouble feeding or has been diagnosed with failure to thrive.