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Clinical manifestations and diagnosis of eosinophilic esophagitis

Authors
Peter A L Bonis, MD
Glenn T Furuta, MD
Section Editor
Nicholas J Talley, MD, PhD
Deputy Editor
Shilpa Grover, MD, MPH

INTRODUCTION

Esophageal eosinophils were once considered to be a hallmark of gastroesophageal reflux disease [1]. However, it has become apparent that the esophagus, which is normally devoid of eosinophils, is an immunologically active organ that is capable of recruiting eosinophils in response to a variety of stimuli [2,3].

When the gastrointestinal eosinophilia is limited to the esophagus, is accompanied by characteristic symptoms, and other causes of eosinophilia have been ruled out, it is termed eosinophilic esophagitis. A panel of experts defined eosinophilic esophagitis as "a chronic, immune/antigen-mediated, esophageal disease characterized clinically by symptoms related to esophageal dysfunction and histologically by eosinophil-predominant inflammation" [4]. In the past, eosinophilic esophagitis was abbreviated as "EE," but because of confusion with erosive esophagitis, many prefer the abbreviation "EoE."

This topic will review the clinical manifestations and diagnosis of eosinophilic esophagitis in adults and children. The genetics, immunopathogenesis, and management of eosinophilic esophagitis; eosinophilic gastroenteritis; and other diseases with eosinophilic involvement of specific organs are discussed separately. (See "Eosinophilic esophagitis (EoE): Genetics and immunopathogenesis" and "Treatment of eosinophilic esophagitis" and "Eosinophilic gastroenteritis" and "Eosinophil biology and causes of eosinophilia", section on 'Disorders with eosinophilic involvement of specific organs'.)

EPIDEMIOLOGY

Eosinophilic esophagitis has been reported in several countries in North and South America, Europe, Asia, and Australia, but there have been no published reports from countries in Africa. A study of a pathology database from the United States found that the disease has been detected in most states that reported data [5]. The results of one survey suggested there may be regional variation, with a higher prevalence in northeastern states and lower prevalence in western states [6]. The diagnosis also appears to be more common in urban as opposed to rural settings. Prevalence within the United States may also differ between climate zones with a higher prevalence in cold and arid zones as compared with the tropical zones [7].

The first cases of probable eosinophilic esophagitis were reported in the late 1960s to 1970s [8-10]. Early reports (mostly from the 1990s) described patients with multiple esophageal rings, which were attributed to gastroesophageal reflux disease or a congenital origin [8,11-16]. The assumed association with gastroesophageal reflux disease (GERD) was based upon the observation that biopsies from patients with a ringed esophagus had basal zone hyperplasia, papillary lengthening, and intraepithelial eosinophils, findings that are seen in patients with documented reflux disease. However, careful review of these reports has raised questions about the association with GERD, since many of the patients did not respond to antisecretory therapy or have objective evidence of reflux on a 24-hour pH study [11,17]. In addition, esophageal dilation in some of these patients was associated with deep mucosal tears and esophageal perforation, complications also seen in patients with eosinophilic esophagitis who undergo dilation [12-14,16,18-22].

                    

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