A 24-year-old man presents to the emergency department (ED) complaining of sudden-onset dysphagia that began 2 hours earlier while he was eating lunch. He states that it feels as though something is “stuck in his throat.” He explains that he tried to drink a glass of water to relieve the feeling but immediately vomited. He is now fearful of eating and drinking. The patient denies any previous episodes of dysphagia and reports no odynophagia, nausea, abdominal pain, heartburn, dyspepsia, weight loss, chest pain, or shortness of breath. He denies a medical history of gastroesophageal reflux disease (GERD) and peptic ulcer disease. He has no known food or drug allergies and is not taking any medications. The patient denies use of alcohol, illicit drugs, and tobacco.
The patient’s vital signs are normal: blood pressure, 118/63 mm Hg; pulse, 95 beats per minute; respiratory rate, 18 breaths per minute; temperature, 97.8 °F; and oxygen saturation, 100%.
On physical examination, the patient is alert and oriented x4. He appears uncomfortable but is in no acute distress. Cardiac examination reveals S1 and S2 heart sounds, with regular rate and rhythm without murmurs. The patient’s lungs are clear to auscultation. His neck is symmetrical with no masses. He has a palpable and nontender thyroid. His trachea is midline and palpable in the suprasternal notch. His abdomen is nondistended and nontender with active bowel sounds. No organomegaly is present.
Initial Diagnosis and Follow-up
No laboratory or diagnostic tests are ordered. Based on the initial assessment, the ED physician diagnoses the patient with GERD and prescribes a liquid proton pump inhibitor (PPI). The patient is hesitant to take the medication for fear of vomiting, but the doctor reassures him that this is not likely to occur.
Twenty minutes after taking the PPI, the patient experiences no improvement of dysphagia. However, because of the patient’s ability to swallow the prescribed medication, the physician determines that no further workup is needed. The patient is sent home and assured that symptoms will resolve soon.
Early the next morning, the patient returns to the ED with unresolved dysphagia. He states that he vomited 30 minutes after leaving the ED. The gastroenterology team is consulted and they perform an esophagogastroduodenoscopy (EGD).
The EGD reveals a food bolus impaction and esophageal stricture located in the distal third of the esophagus. Mild esophagitis also is noted throughout the esophagus. Given these findings, biopsies are obtained for histology. The results indicate a diagnosis of eosinophilic esophagitis (EoE).
During the initial EGD, the food impaction is removed and the esophagus is treated with dilation. The patient is discharged home with a prescription for omeprazole 40 mg daily and is referred to an allergist.
Allergy testing reveals allergic reactions to dairy, shellfish (shrimp, crab, lobster, clams, mussels, oysters, and scallops), bananas, and sweet potatoes. The allergist suggests diet modification and corticosteroids as treatment options. Given the potential long-term side effects of corticosteroids, the patient decides that an elimination diet and a daily PPI is the best treatment option for him.
Two years after being diagnosed with EoE, the patient returns to the gastroenterologist after experiencing another episode of dysphagia. Repeat EGD reveals no stricture but shows diffuse faint corrugated mucosa throughout the esophagus, suggesting worsening disease. Further questioning of the patient reveals that the esophageal findings and recurrent symptoms likely are because of his nonadherence with his diet plan.
Eosinophilic esophagitis is a chronic immune-mediated disease that is increasingly encountered in clinical practice.1 The disease is characterized by infiltration of eosinophils and other inflammatory cells into the esophageal epithelium.1 This causes tissue remodeling and fibrosis of the esophagus, leading to symptoms of esophageal dysfunction. A strong correlation has been found between the development of EoE in patients with a personal or family history of atopic disease, specifically asthma, eczema, and food allergies.1 This correlation makes it important to obtain a detailed history to identify possible risk factors for developing EoE. In this case, the patient presented with no medical history of atopic disease, which made this a more challenging diagnosis.
A wide variety of symptoms are associated with EoE. Adults can present with dysphagia, heartburn, food impaction, chest pain, odynophagia, and vomiting.1,2 Although these symptoms are common in patients with EoE, they are not exclusive to the disease and can occur with other gastrointestinal disorders.
Differentiating EoE and GERD
Gastroesophageal reflux disease occurs when the lower esophageal sphincter relaxes, allowing reentry of acid and food into the esophagus, which causes injury and inflammation.3 Patients with GERD can present with symptoms of heartburn, dysphagia, chest pain, reflux, abdominal pain, and nausea.3 The similarity between symptoms of EoE and GERD makes distinguishing these 2 diseases clinically challenging (Table).1-3
The patient in this case presented with dysphagia, a common symptom found in patients with both diseases, and was initially misdiagnosed with GERD. The prevalence of EoE is significantly lower than that of other esophageal diseases, occurring in 57 per 100,000 people.4 The lower prevalence and the nonspecific symptoms associated with EoE may contribute to its frequent misdiagnosis.
The diagnosis of EoE is based on clinical presentation, histological findings, and the exclusion of other gastrointestinal diseases.3 The diagnosis of EoE should be considered in patients presenting with clinical symptoms of esophageal dysfunction, such as dysphagia, heartburn, chest pain, strictures, and food impactions, and an EGD should be considered to view the mucosa and collect multiple biopsies. In patients with EoE, these biopsies commonly will reveal eosinophilia with 15 or more eosinophils per high-power field (hpf).1,3 Patients with suspected EoE should be given a trial of high-dose PPIs for 8 to 12 weeks followed by repeat EGD. If the patient is still symptomatic and biopsies reveal more than 15 eosinophils per hpf, then a diagnosis of EoE can be established.3 If both symptoms and esophageal eosinophilia improve after the PPI trial, then other disorders such as GERD or PPI-responsive esophageal eosinophilia (PPI-REE) should be considered.
Differentiating EoE From PPI-REE
Proton pump inhibitor-responsive esophageal eosinophilia is a relatively new disorder that is genetically and phenotypically indistinguishable from EoE.5,6 Although PPI-REE can be clinically and histologically identical to EoE, patients with PPI-REE can achieve complete remission after high dose-PPI therapy.3 Given the similarities between EoE and PPI-REE, it is important that all patients with suspected EoE be given a PPI trial to rule out PPI-REE. Patients with PPI-REE should continue PPI therapy, tapered down to the lowest possible dose depending on clinical symptoms.5 Follow-up endoscopy should be performed 1 year after diagnosis to ensure continued histological remission.5
Referral for Allergy Testing in EoE
Once a diagnosis of EoE has been established, patients should be referred to an allergist for testing and determination of food allergens.1 When food triggers are identified and removed from the diet, patients should experience an improvement of symptoms.1 Common diet modifications include the targeted elimination and empiric 6-food elimination diets.1 The targeted elimination diet uses skin patch testing to determine which foods need to be removed from the diet. The empiric 6-food elimination diet focuses on eliminating major food allergens, such as milk, eggs, wheat, soy, peanut and tree nuts, and all seafood, from the diet.7 Although both of these dietary modification strategies are very effective, it is important that providers ensure that patients adhere to the diet. The patient in this case was prescribed a targeted elimination diet but experienced a recurrence of symptoms due to nonadherence.
For patients who are nonadherent or whose symptoms are not well controlled with diet alone, providers should consider initiating corticosteroid therapy, such as fluticasone and budesonide.6 Although they are not indicated for EoE, corticosteroids are useful in the resolution of EoE symptoms because of their ability to reduce inflammation in the esophagus and suppress eosinophil production.6 Corticosteroids can be prescribed in a liquid or nebulized form. Nebulized corticosteroids are puffed into the mouth and swallowed, not inhaled, to coat the entire esophagus. Educating patients about how to appropriately use these medications is vital to ensure therapeutic efficacy. It also is necessary to inform patients about the possible side effects of swallowed steroid therapy, such as dry mouth, local candida infections, adrenal axis suppression, glaucoma, and bone demineralization.6
Along with diet modification, this patient was prescribed a daily PPI for the management of EoE. Although PPIs are not considered first-line therapy, they may be prescribed for symptomatic treatment. These medications can help reduce acid production, providing relief from heartburn, regurgitation, and dysphagia.6 Although PPIs are helpful in the resolution of these symptoms, they are also effective in treating the underlying inflammatory process and can be used as monotherapy.4
In some cases, patients with EoE can suffer from esophageal strictures and food impactions. Esophageal dilation is used for the immediate relief of symptoms in these patients.1 Understanding when dilation should be performed is an important aspect of managing patients with EoE.8
Karmen Elsen, MPA, PA-C, works in the Digestive Health Center at Augusta University in Augusta, Georgia.
- Merves J, Muir A, Chandramouleeswaran PM, Cianferoni A, Wang ML, Spergel JM. Eosinophilic esophagitis. Ann Allergy Asthma Immunol. 2014;112(5):397-403. doi:10.1016/j.anai.2014.01.023
- Park H. An overview of eosinophilic esophagitis. Gut Liver. 2014;8(6):590-597. doi:10.5009/gnl14081
- Kia L, Hirano I. Distinguishing GERD from eosinophilic oesophagitis: concepts and controversies. Nat Rev Gastroenterol Hepatol. 2015;12(7):379-386. doi:10.1038/nrgastro.2015.75
- Dellon ES, Jensen ET, Martin CF, Shaheen NJ, Kappelman MD. Prevalence of eosinophilic esophagitis in the United States. Clin Gastroenterol Hepatol. 2014;12(4):589-596.e1. doi:10.1016/j.cgh.2013.09.008
- Molina-Infante J, Rodriguez-Sanchez J, Martinek J, et al. Long-term loss of response in proton pump inhibitor-responsive esophageal eosinophilia is uncommon and influenced by CYP2C19 genotype and rhinoconjunctivitis. Am J Gastroenterol. 2015;110(11):1567-1575. doi:10.1038/ajg.2015.314
- Furuta GT, Katzka DA. Eosinophilic esophagitis. N Engl J Med. 2015;373(17):1640-1648. doi:10.1056/NEJMra1502863
- Greenhawt M, Aceves S. Non-IgE medicated food allergy: eosinophilic esophagitis update on the pathogenesis, clinical features, and management of eosinophilic esophagitis in children. Cur Ped Reports. 2014;2(2):127-134.
- Al-Hussaini A. Savary dilation is safe and effective treatment for esophageal narrowing related to pediatric eosinophilic esophagitis. J Pediatr Gastroenterol Nutr. 2016;63(5):474-480. doi:10.1097/MPG.0000000000001247