Correspondence
Eosinophilic Esophagitis
N Engl J Med 2004; 351:940-941August 26, 2004
- Article
To the Editor:
Eosinophilic esophagitis is an emerging disease worldwide, as documented by recent case series from Switzerland, Australia, Italy, Spain, Japan, England, and the United States.1,2 Eosinophilic esophagitis mimics gastroesophageal reflux disease and may result in narrowing and stricture of the esophagus.1-3 This disease is differentiated from reflux esophagitis on the basis of the magnitude of mucosal eosinophilia and a lack of response to acid suppression.4 We report findings from a population-based demographic study of the pediatric population with eosinophilic esophagitis residing in the vicinity of our medical center (Hamilton County, Ohio), a region with a single pediatric gastroenterology and pathology provider.
Cases of eosinophilic esophagitis were systematically identified from our institution's pathology database; the criteria were the presence of epithelial proliferative changes (e.g., thickening of the basal epithelial layer and elongation of the papillae), a minimum of 24 eosinophils per high-power field (×400) in the distal esophagus, and the absence of eosinophilia in any other intestinal segment. Data from the 2000 Census for the population 0 to 19 years of age were used to calculate frequency.
During the years 1991 through 2003, there were 924 possible cases of eosinophilic esophagitis, of which 315 were subsequently found to meet the diagnostic criteria. Of the 315 cases, only 2.8 percent had been identified before 2000. A total of 103 patients with conditions that met the histologic criteria for the disease resided within Hamilton County at the time of diagnosis. More than 70 percent of these 103 patients had coexisting eosinophilic involvement in the proximal esophagus. Approximately 71 percent were male, with a mean (±SD) age of 10.5±5.4 years. The patients presented with the typical symptoms and atopic history that have been described previously (Table 1Table 1
Presenting Symptoms among 103 Pediatric Patients with Eosinophilic Esophagitis. and Table 2Table 2
History of Atopy in the 103 Pediatric Patients.).1-4 However, our demographic analysis revealed a strong familial pattern (Table 2), including three sibling pairs; the mother of one of the pairs of siblings was also given a diagnosis of eosinophilic esophagitis at our institution. The familial cases of eosinophilic esophagitis, diagnosed according to the histologic criteria applied at our institution, are included in the case totals.For the years 2000 through 2003, the annual incidence of eosinophilic esophagitis in the pediatric population for Hamilton County was approximately 1 per 10,000, with a prevalence of 4.296 cases per 10,000 children at the end of 2003 (Table 3Table 3
Frequency Estimate for Eosinophilic Esophagitis According to Age, 2000–2003.). Similar rates were observed for specific age groups (birth to 4, 5 to 9, 10 to 14, and 15 to 19 years). Analysis of the population residing within a 10-mile radius of our medical center produced similar results. Cases of eosinophilic esophagitis were distributed along the population-density gradient, rather than clustered in particular geographic regions (Spearman correlation factor, 0.59; P<0.001). The chi-square test for trend among all cases within the Hamilton County population during our four-year study was not significant (P=0.23). Our frequency estimates should be considered a minimum, since they do not account for unrecognized cases.Our data establish values for the incidence of eosinophilic esophagitis that may be higher than those for other well-recognized inflammatory gastrointestinal disorders (e.g., Crohn's disease).5 Despite this result, there is currently a paucity of information about eosinophilic esophagitis, and many practitioners have not recognized its prevalence. In addition, our finding of a familial pattern suggests two possibilities: first, a genetic predisposition and, second, a cause related to an unknown environmental exposure (e.g., infection or allergen). Both these possibilities now warrant pursuit.
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Philip E. Putnam, M.D.
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