Study reveals long-term outcomes for children with eosinophilic esophagitis (EoE)

Important article from Cincinnati Children's Hospital: http://www.cincinnatichildrens.org/health/subscribe/ped-insights/06-11/eosinophilic.htm

June 2011
Study reveals long-term outcomes for children with eosinophilic esophagitis (EoE)

The first study of its kind to examine the long-term natural history of eosinophilic esophagitis (EoE) reveals that children with EoE who also have other allergies are most likely to suffer severe complications from the disease. They also face higher risk of symptoms lasting years into adulthood.

The study, published online this month in the Journal of Allergy and Clinical Immunology, was conducted by researchers at Cincinnati Children’s Hospital Medical Center who studied hundreds of people diagnosed with esophageal eosinophilia before EoE was known to be a separate disease. The study’s lead authors were James Franciosi, MD, MS, MSCE, and Charles DeBrosse, MD.

“This important set of findings suggests that disease severity is greater among patients with allergies,” says Marc Rothenberg MD, PhD, director of the Division of Allergy and Immunology and the Cincinnati Center for Eosinophilic Disorders, and senior author of the study.

“Taken one step further, these data support the need for allergy testing among patients with EoE, as identifying patients susceptible to allergic reactions will not only assist in dietary management, but will help identify those at greatest risk for persistent disease,” Franciosi says.

The study concludes that esophageal eosinophilia is associated with reduced quality of life and persistent symptoms as much as 15 years after presentation. In addition, elevated eosinophil counts in combination with other allergies during childhood increase the risk of swallowing difficulties in adulthood.

What is EoE?

Eosinophilic esophagitis is an inflammation of the esophagus that results in many symptoms, including difficulty swallowing (dysphagia) as well as structural changes and high eosinophil counts (eosinophilia), a type of white blood cell typically involved in allergy and parasitic infections. Sometimes this inflammatory reaction is so severe that managing symptoms requires eliminating solid food and providing nutrition via special liquid formula.

EoE was established as a separate disease in 2007; defined as at least 15 eosinophils in a high-powered field (HPF) of esophageal tissue. Until recently, patients suffering from this disease were often misdiagnosed as having gastroesophageal reflux disease (GERD) or chronic esophagitis.

Researchers now estimate that about one in every 1,000 people in the U.S. suffer from EoE. Cincinnati Children’s encounters about 30 cases each week.

“This emerging disorder is attracting increasing interest among allergists, gastroenterologists, pathologists, and primary care physicians,” Rothernberg says. “However, identifying how best to manage this condition has been hindered by a lack of data regarding long-term outcomes.”

Study method

Researchers at Cincinnati Children’s embarked upon a novel project to examine the natural history of EoE during the transition from childhood into adulthood.

The project involved reviewing 3,817 pediatric esophageal biopsy specimens from 1982-1999. This revealed more than 660 patients with retrospectively identified eosinophilic esophagitis (rEoE) and chronic esophagitis (CE). This group, as well as an age-matched control group, were asked to complete health-related outcome questionnaires.

The study collected 209 completed questionnaires.

Key findings

The surveys show that quality of life was significantly decreased among patients with rEoE and CE compared to the control group. In fact, 73 percent of patients with EoE suffered symptoms into adulthood.

Dysphagia was reported among 49 percent of patients with rEoE and 37 percent of patients with CE compared to 6 percent for the control group. Elevated eosinophil counts during childhood were predictive of this condition in adulthood.

Food impaction was reported among 40 percent of patients with rEoE and 14 percent of patients with CE compared to 3 percent of the control group.

Higher rates of dysphagia occurred if EoE patients also had a food allergy, allergic rhinitis or asthma. Food impaction was more common among patients with reported food allergy.

Next steps

This paper reports the longest natural history study of pediatric EoE to date.

“It is rare to have this type of information on patients for any disease, mainly because the patients went untreated for such a long period of time,” Rothenberg says. “In this case, patients did not know they had the disease.”

This study establishes that rising levels of esophageal eosinophils correlate with worsening disease symptoms. Notably, even though the diagnosis of EoE is currently reserved for patients that have at least 15 eosinophils/HPF, the study reports persistent symptoms associated with levels as low as five eosinophils/HPF.

These findings support more comprehensive allergy testing for patients with EoE to help guide symptom management. In addition, the evidence of long-lasting symptoms for untreated EoE substantiates more aggressive treatment approaches for children.

“Esophageal eosinophilia should not be ignored,” Rothenberg says. “Presence of food allergy with evidence of esophageal eosinophilia should prompt clinicians for more careful clinical attention and treatment and follow up.”

For more information about EoE and its treatment, contact the Cincinnati Center for Eosinophilic Disorders at 513-636-2233 (CCED) or 1-800-344-2462, ext. 6-2233; or email us at cced@cchmc.org.