Clinical presentation of patients with eosinophilic inflammation of the esophagus

doi:10.1016/j.gie.2006.03.931

Gastrointestinal Endoscopy

Volume 64, Issue 4, October 2006, Pages 473-478

https://doi.org/10.1016/j.gie.2006.03.931 Get rights and content

Background

Allergic eosinophilic esophagitis (AEE) is defined by a dense infiltrate of eosinophils within the esophageal mucosa and the absence of pathological gastroesophageal reflux.

Objective

To characterize a pediatric population with AEE to determine if AEE can be diagnosed based on history; to compare patients with varying degrees of esophageal eosinophilic inflammation to determine if moderate esophageal inflammation is part of a continuum of AEE.

Design

Medical records of 112 patients with eosinophils on esophageal biopsy specimens were reviewed. Patients were grouped according to eosinophils per high power field (eos/hpf): group 1 (1-5 eos/hpf, n = 31), group 2 (6-14 eos/hpf, n = 13), and group 3 (≥15 eos/hpf, n = 68) and compared.

Setting

University Children's Hospital.

Patients

Children and adolescents with eosinophils on esophageal mucosal biopsy specimens.

Interventions

Analysis of clinical information.

Main Outcome Measurements

Clinical characterization of patients with esophageal eosinophilia.

Results

There was no significant difference in patient demographics. Patients in groups 2 and 3 had multiple food allergens identified. Patients in group 3 with a positive type I allergic response were significantly younger than those with a negative response (mean, 4.6 years old vs mean, 8.5 years old; P = .0065). In group 2, 3 of 13 patients responded histologically to acid-suppressive therapy, whereas 6 patients had improved histology with corticosteroids; 4 of these 6 patients had not responded histologically to acid-suppression.

Limitations

Retrospective study.

Conclusions

History and clinical presentation were not useful in predicting the severity of histologic esophageal inflammation in this cohort. Patients with moderate esophageal eosinophilia (group 2) exhibited a variable response to medical therapy.

Section snippets

Patients

The study was conducted at the James Whitcomb Riley Hospital for Children, Indiana University, Indianapolis, Indiana. After obtaining approval from the Indiana University Institutional Review Board, medical records of patients ≥12 months of age, seen between January 2000 and January 2001, with eosinophils in esophageal mucosal biopsy specimens were reviewed. A physician collected demographic and clinical information that included age; sex; symptoms; allergy history; total serum immunoglobulin

Results

During the study period, 154 patients had ≥1 eosinophil on esophageal mucosal biopsy specimens. Of these, 112 patients met the inclusion criteria. The mean age was 7.5 years (median, 6.9 years; range, 1-21.1 years) and 74 were male (66%). There were 31 patients in group 1, 13 in group 2, and 68 in group 3.

No statistically significant differences in age, sex, or symptoms were noted among the groups, though chest pain/heartburn was more common in group 1, and dysphagia was more common in groups 2

Discussion

The presence of eosinophils within the esophageal mucosa is pathologic.¹⁰ While esophageal eosinophilia can be present in a variety of diseases, including inflammatory bowel disease and collagen vascular disease, the most common inciting events encountered in clinical practice are thought to be GER and, more recently, AEE.10, 17 GER results in only a few esophageal eos/hpf, whereas AEE is associated with dense eosinophilic infiltrates.5, 6, 7, 10, 18

Because of the increasing recognition and

Acknowledgments

The authors are thankful for the assistance and the insight provided by Mary Davis, MD (James Whitcomb Riley Hospital for Children, Division of Pediatric Pathology), Gilbert C. Liu, MD, MS (Indiana University School of Medicine, Children's Health Services Research), and Luke Clapp. Lori Heady provided secretarial assistance.

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The incidence and prevalence of eosinophilic esophagitis (EoE) have markedly increased over the past 2 decades, outpacing increased detection of the disease. Although genetic susceptibility markers for EoE have begun to be elucidated, the rate at which EoE has increased in incidence suggests environmental factors predominate. Despite many advances in understanding of the pathogenesis of EoE, the cause of EoE is unknown. This article reviews the emerging data related to environmental risk factors for EoE. Many of these environmental factors are rooted in the theoretical framework of the hygiene hypothesis, specifically mediation of disease development through dysbiosis. Other hypotheses are based on associations that have been observed in studies of non-EoE allergic disease. We describe the evidence that early-life exposures, including antibiotic use, acid suppression, and cesarean delivery, can increase the risk of disease. We also describe the evidence that infectious agents, such as Helicobacter pylori, are inversely associated with disease. Current evidence on geographic risk factors, such as population density, climate zone, and seasonality, is reviewed. We also describe behavioral factors that have been evaluated. Limitations of the existing research are discussed, and recommendations for future areas of research, including assessment of gene-environment interaction, are presented.
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This retrospective cohort study from 2001 to 2014 included subjects with a new diagnosis of EoE per consensus guidelines. Demographic, endoscopic, histologic, and treatment response data were extracted from medical records. An extremely narrow-caliber esophagus was defined when a neonatal endoscope was required to traverse the esophagus due to the inability to pass an adult endoscope. Patients with and without an extremely narrow-caliber esophagus were compared. Multivariable logistical regression was performed to assess treatment outcomes.
Of 513 patients with EoE, 46 (9%) had an extremely narrow-caliber esophagus. These patients were older (33 vs 22 years; P < .01), had longer symptom duration (11 vs 3 years; P < .01), more dysphagia (98% vs 66%; P < .01), and food impactions (53% vs 31%; P < .01). Dilation was more common with extreme narrowing (69% vs 17%; P < .01). Patients with a narrow-caliber esophagus were more refractory to steroid treatment, with lower symptom (56% vs 85%), endoscopic (52% vs 76%), and histologic (33% vs 63%) responses (P < .01 for all), and these differences persisted after multivariate analysis.
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Eosinophilic oesophagitis (EoE) has first been described a little over 20 years ago. EoE has been defined by a panel of international experts as a “chronic, immune/antigen-mediated, oesophageal disease, characterized clinically by symptoms related to oesophageal dysfunction and histologically by eosinophil-predominant inflammation”. A value of ≥15 eosinophils has been defined as histologic diagnostic cutoff. Other conditions associated with oesophageal eosinophilia, such as gastro-oesophageal reflux disease (GERD), PPI-responsive oesophageal eosinophilia, or Crohn's disease should be excluded before EoE can be diagnosed. This review highlights the latest insights regarding the diagnosis and differential diagnosis of EoE.
Pediatric Eosinophilic Esophagitis Symptom Scores (PEESS v2.0) identify histologic and molecular correlates of the key clinical features of disease
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We systematically recruited treated and untreated pediatric patients with EoE (aged 2-18 years) and examined parent proxy–reported symptoms using the PEESS v2.0. Clinical symptomology was collected by questionnaire. Esophageal biopsy samples were quantified for levels of eosinophils, eosinophil peroxidase (EPX) immunohistochemical staining, and mast cells. Molecular features were assessed by using the EoE Diagnostic Panel (94 EoE-related gene transcripts). Associations between domain scores and clinical symptoms and biological features were analyzed with Wilcoxon rank sum and Spearman correlation.
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Original ArticleClinical EndoscopyClinical presentation of patients with eosinophilic inflammation of the esophagus

Background

Objective

Design

Setting

Patients

Interventions

Main Outcome Measurements

Results

Limitations

Conclusions

Section snippets

Patients

Results

Discussion

Acknowledgments

J Allergy Clin Immunol

J Allergy Clin Immunol

Gastroenterology

Gastroenterology

Am J Gastroenterol

J Allergy Clin Immunol

Clin Gastroenterol Hepatol

Eosinophilic esophagitis in children and adults

J Pediatr Gastroenterol Nutr

An etiological role for aeroallergens and eosinophils in experimental esophagitis

J Clin Invest

Primary eosinophilic esophagitis in children: successful treatment with oral corticosteroids

J Pediatr Gastroenterol Nutr

Original Article
Clinical Endoscopy
Clinical presentation of patients with eosinophilic inflammation of the esophagus