Eosinophilic Esophagitis
Overview
Eosinophilic esophagitis is a chronic condition that causes inflammation of the esophagus, the tube connecting the mouth to the stomach. The condition can cause trouble swallowing, chest pain, vomiting, abdominal pain, and other symptoms in both children and adults. Eosinophilic esophagitis is becoming more common, especially among people with allergies or asthma. With the right treatment, most people with eosinophilic esophagitis can manage symptoms and maintain a good quality of life.
What is eosinophilic esophagitis?
Eosinophilic esophagitis, also called EoE, is a chronic immune-mediated condition that causes inflammation in the esophagus. Immune-mediated means the immune system reacts to certain foods or substances in a way that damages healthy tissue.
In eosinophilic esophagitis, an allergic reaction to certain foods or other substances causes a type of white blood cell, called an eosinophil, to build up in the esophageal lining where it is not usually found. This buildup of eosinophils leads to swelling, irritation, and sometimes scarring of the esophagus.
Eosinophilic esophagitis affects both children and adults. It is often diagnosed in childhood or young adulthood. It is one of the most common causes of swallowing problems in young adults. The condition can cause a range of symptoms, including difficulty swallowing, food avoidance, chest pain, abdominal pain, vomiting, reflux, and heartburn. Eosinophilic esophagitis is more likely to occur in people who have other allergic conditions, such as asthma, eczema (a skin condition causing itchy rashes), or hay fever (allergic rhinitis).
Eosinophilic esophagitis is a lifelong condition that requires ongoing management.
What causes eosinophilic esophagitis?
The causes of eosinophilic esophagitis are not fully understood, but the condition is believed to result from a combination of immune, genetic, and environmental factors. Eosinophilic esophagitis is considered an allergic disease. In the condition, the immune system reacts to certain triggers—often foods or substances in the environment—by sending eosinophils to the esophagus where they build up and cause symptoms.
Many people with eosinophilic esophagitis also have other allergic disorders, such as asthma or eczema. In people with these disorders, the immune system may be more likely to react to common foods or airborne allergens such as pollen, dust mites, or mold. In eosinophilic esophagitis, exposure to certain triggers causes the esophagus to become inflamed and swollen.
Because pollen and other environmental allergens can make eosinophilic esophagitis worse, it is more common for people to experience symptoms when pollen or other allergen counts are high, usually in spring, summer, or fall.
Genetic factors can also play a role. Eosinophilic esophagitis can run in families, and certain genes involved in immune responses have been linked to the disease. For example, changes in genes that control immune cell activity and inflammation may increase the risk of developing eosinophilic esophagitis.
Other conditions can cause high eosinophil levels in the esophagus. For example, gastroesophageal reflux disease (GERD), parasitic infections, and inflammatory bowel disease can all result in high eosinophil levels in esophageal tissue. However, in eosinophilic esophagitis, inflammation is usually limited to the esophagus and is not caused by acid reflux alone.
Eosinophilic esophagitis is thought to be caused by an abnormal immune response to foods or environmental allergens in people who are genetically susceptible, but the exact triggers can vary from person to person.
What are the risk factors for eosinophilic esophagitis?
Risk factors for eosinophilic esophagitis include a combination of personal, family, and environmental factors. The main risk factors are:
- Male sex: Eosinophilic esophagitis affects about three times as many men as women.
- History of allergies and asthma: People with food or environmental allergies, asthma, eczema, or other chronic allergic conditions are more likely to develop eosinophilic esophagitis.
- Family history: Having a close relative with eosinophilic esophagitis increases the risk.
- Early life exposures: Some studies suggest that early antibiotic use, formula feeding, or cesarean delivery (C-section) may increase risk, but these links are not fully understood.
What are the symptoms of eosinophilic esophagitis?
Symptoms of eosinophilic esophagitis vary by age and may range from mild to severe. The most common symptoms include trouble swallowing and food getting stuck in the esophagus, but other signs can also occur.
In infants and toddlers, symptoms may include:
- Feeding problems: Babies may refuse to eat or have trouble nursing or taking bottles.
- Growth faltering: Infants may not gain weight as expected.
- Regurgitation and/or vomiting: Babies may spit up frequently or may not respond to typical treatments for reflux.
In children, symptoms may include:
- Difficulty swallowing: Children may eat slowly or avoid certain foods.
- Chest and abdominal pain: Children may complain of chest pain and stomach aches.
- Poor appetite: Children may not want to eat or lose interest in food.
- Vomiting: Some children may throw up after eating.
In adolescents and adults, symptoms may include:
- Difficulty swallowing: Food may feel like it is stuck in the throat or chest.
- Food impaction: Food can become lodged in the esophagus, sometimes requiring emergency removal.
- Heartburn: Some people feel burning in the chest, similar to acid reflux.
- Chest pain or upper abdominal pain: Pain may occur after eating or at other times.
- Regurgitation or backflow of undigested food: Food or liquid may come back up into the mouth, even hours after eating.
How is eosinophilic esophagitis diagnosed?
Diagnosis of eosinophilic esophagitis involves a careful review of symptoms, medical history, and a physical exam, followed by specific tests to confirm the condition and rule out other causes.
Doctors begin by asking about swallowing problems, chest pain, heartburn, and personal or family history of allergies, asthma, or eczema. They may also ask about previous treatments for reflux or other digestive problems.
The physical exam usually doesn’t show anything abnormal, and the doctor may check for signs of allergies and abdominal tenderness.
To confirm the diagnosis and rule out other conditions, doctors may order the following tests:
- Upper endoscopy (also called esophagogastroduodenoscopy, EGD): A doctor uses a thin, flexible tube with a camera to look at the esophagus for signs of inflammation, rings, furrows, or narrowing. However, in some cases of eosinophilic esophagitis, the esophagus may appear normal.
- Biopsy: During endoscopy, the doctor takes small samples of the esophageal lining to examine under a microscope to look for eosinophils and signs of inflammation. This is a necessary step for diagnosis of eosinophilic esophagitis.
- Blood tests: Various blood tests can help rule out other causes of symptoms or check for signs of allergic disease.
- Barium swallow: For this X-ray test, the person swallows a special liquid that highlights the esophagus and shows narrowing or rings that may indicate eosinophilic esophagitis.
A diagnosis of eosinophilic esophagitis is made when at least 15 eosinophils per high-powered field are seen on biopsy, and other causes of esophageal eosinophilia, such as acid reflux or infections, are ruled out.
How is eosinophilic esophagitis treated?
Treatment for eosinophilic esophagitis focuses on reducing inflammation, managing symptoms, and preventing long-term damage. There is no cure, but several therapies can help control the disease.
Treatments may include:
- Dietary changes: Many people can reduce symptoms by avoiding foods suspected of causing symptoms. The six most common trigger foods include cow’s milk, wheat, eggs, soy, nuts, and seafood. Two systematic diets can help identify food triggers:
- Elimination diets remove common triggers and other suspected foods for several weeks, then reintroduce them one at a time to see what triggers a reaction.
- In an elemental diet, a special formula with amino acids replaces regular food for several weeks, then other foods are reintroduced one at a time.
- Acid-blocking medicines: Proton pump inhibitors (PPIs), which reduce the amount of acid in the digestive tract, can reduce inflammation and help with reflux symptoms. They are often tried first to reduce symptoms.
- Topical corticosteroids: In some cases, swallowed steroids (such as fluticasone or budesonide, and occasionally prednisone) can help reduce inflammation in the esophagus.
- Monoclonal antibodies: Newer treatments, such as monoclonal antibodies (for example, dupilumab), target specific parts of the immune system and may be used if other therapies do not work. These are administered by injection based on age and/or weight.
- Esophageal dilation: If the esophagus becomes narrowed or scarred, a doctor may stretch it during an endoscopy to make swallowing easier. This is usually done if other treatments do not help.
Doctors may recommend a combination of these treatments, depending on symptoms and test results. Ongoing follow-up is important to check for improvement and adjust therapy as needed.
What are the potential complications of eosinophilic esophagitis?
Complications of eosinophilic esophagitis include changes to the structure of the esophagus and difficulty swallowing and eating foods.
- Esophageal stricture: Narrowing or scarring of the esophagus can make swallowing difficult and may require stretching procedures.
- Food impaction: Food can become stuck in the esophagus, sometimes causing pain or requiring emergency removal.
- Damage to the esophagus: Forceful vomiting or untreated food impaction can cause tears or holes, which need immediate medical attention.
- Ongoing inflammation: Long-term swelling can lead to fibrosis (scarring) and permanent changes in the esophagus.
Ongoing treatment and monitoring can help reduce complications. People should seek emergency care if they have severe chest pain, trouble breathing, or cannot swallow food or liquids.
What is the outlook for people with eosinophilic esophagitis?
The outlook for people with eosinophilic esophagitis is generally good with proper treatment. Although eosinophilic esophagitis is a chronic condition that requires ongoing care, most people can manage symptoms and maintain a normal lifestyle.
Early diagnosis and following the recommended treatment plan can improve outcomes. Untreated eosinophilic esophagitis can lead to long-term complications, such as strictures or repeated food impactions. With regular follow-up and the right therapy, most people experience relief from symptoms and avoid serious problems.
What stands out about Yale Medicine's approach to eosinophilic esophagitis?
“Eosinophilic esophagitis is a serious condition that is becoming more common, and patients need providers that are familiar with how to diagnose and treat the condition,” says Alex Koral, MD, a Yale Medicine pediatric gastroenterologist who treats a variety of issues including eosinophilic esophagitis. “The Eosinophilic Esophagitis and Gastrointestinal Diseases Program focuses on comprehensive care for patients with eosinophilic esophagitis and other eosinophilic diseases in the GI tract. The providers in the program work closely with dietitians and nurses to ensure comprehensive clinical and nutritional support, while also working closely with colleagues in allergy, dermatology, and pulmonology to provide comprehensive medical care. The gastroenterologists in the program are experts in the field, with familiarity in the latest diagnostic tools and treatments, as well as collaborating with other eosinophilic esophagitis experts from around the country.”
Yale also features one of the first pediatric transnasal endoscopy programs in the country, which is a way to biopsy the esophagus without anesthesia. “Yale is an ideal place to receive up-to-date, comprehensive and compassionate care for eosinophilic esophagitis,” adds Dr. Koral.