In the absence of pathologic gastroesophageal reflux disease, young children with esophageal symptoms or feeding problems and older children and adults with dysphagia or esophageal food impaction should be evaluated for eosinophilic esophagitis, particularly if their symptoms are unresponsive or only partially responsive to acid blockade, according to a new consensus report on the inflammatory gastroesophageal disorder. The report appears in the October 2007 issue of Gastroenterology.
Characterized by a range of gastrointestinal symptoms including abdominal pain, nausea, regurgitation, and/or vomiting along with severely elevated levels of eosinophils, eosinophilic esophagitis (EE) is an “enigmatic” disease that is frequently mistaken for gastroesophageal reflux disease (GERD), said Dr. Glenn T. Furuta, cochair of the First International Gastrointestinal Eosinophilic Research Symposium (FIGERS), from which the consensus report was generated.
Because EE and GERD require different treatment strategies and are associated with different long-term consequences, distinguishing between the two is critical, but doing so has been hindered by the absence of definitive diagnostic and treatment guidelines for EE, said Dr. Furuta of Children's Hospital of Denver. “The consensus report is the best first step in providing outstanding care to children and adults who are affected by this newly recognized disease,” he said in an interview.
Developed through the collaboration of 32 physicians from multiple disciplines, including pediatric and adult gastroenterology, allergy, and pathology, the consensus report is based on a systematic review of data from 80 studies published between 1977 and 2006 including 754 children and 323 adults, as well as expert opinion.
Among the most important features of the report are its diagnostic guidelines, Dr. Furuta said. In this regard, “the report states that affected patients should have symptoms referable to the esophagus that occur in combination with greater than or equal to 15 eosinophils per high-powered field in the esophageal mucosa with normal gastric and duodenal mucosa,” he said. “Most importantly, GERD must have been ruled out as a cause of these findings.”
The available data on the natural history of the disease suggest that EE is a chronic condition with persistent or relapsing symptoms. “To date, esophageal strictures and small caliber esophagus, often resulting in food impaction, have been the major complications identified,” the authors wrote in the report. “When these findings are encountered, either radiologically or at endoscopy, a high index of suspicion should be raised for EE and mucosal biopsies should be obtained.”
Importantly, mucosal pinch biopsies are recommended for all patients in whom eosinophilic esophagitis is in the differential diagnosis, regardless of the gross appearance of the mucosa, the authors wrote. Additionally, multiple biopsies should be obtained from different esophageal locations along the length of the esophagus, and they should be obtained from the stomach and duodenum to rule out other gastrointestinal diseases, they stated.
When endoscopy and biopsy do not provide sufficient information to distinguish between GERD and eosinophilic esophagitis, “intraesophageal pH monitoring may be of use in excluding pathologic reflux as either the primary or a concomitant cause for esophageal eosinophilia,” according to the report. “Alternatively, an upper endoscopy after 6–8 weeks of high-dose proton pump inhibitor treatment can help determine the etiology of esophageal eosinophilia.”
Regarding diagnostic histopathology, based on the available data and the panel's collective clinical experience, “we conclude that a peak count of 15 intraepithelial eosinophils per high-powered field is an absolute minimum number to make the diagnosis of EE in the proper clinical context,” they wrote. For research purposes, it may be useful to use a higher threshold of peak eosinophils in order to increase the specificity of the diagnosis, they noted.
Additional histopathologic features that, while not pathognomonic, may be helpful in establishing a diagnosis, are eosinophil microabscesses, surface layering of eosinophils, basal layer hyperplasia, papillary lengthening, degranulating eosino- phils, and lamina propria fibrosis and inflammation, according to the report.
In terms of treating the disease, dietary therapy in particular should be considered in all children diagnosed with the condition, as there is strong circumstantial evidence that food allergens contribute to the pathogenesis of the disease in children. In fact, “the removal of food antigens has clearly been demonstrated to successfully treat both the symptoms and the underlying histopathology in the majority of patients with EE,” the authors wrote. Toward this end, allergy testing and clinical history can help guide specific food elimination, they stated, adding that consultation with a registered dietitian is advisable to ensure that proper nutrition is maintained.
Corticosteroids can effectively resolve acute clinicopathologic features of EE, but the disease typically recurs when the steroids are stopped. And while systemic corticosteroids have a role in emergent cases, including dysphagia requiring hospitalization, dehydration because of swallowing difficulties, and weight loss, the potential for significant toxicity over time should preclude their long-term use, the authors stated. Topical corticosteroids have also shown some efficacy and are associated with fewer side effects, they noted.