Review
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World J Gastroenterol. Aug 14, 2014; 20(30): 10419-10424
Published online Aug 14, 2014. doi: 10.3748/wjg.v20.i30.10419
Proton pump inhibitor responsive esophageal eosinophilia, a distinct disease entity?
William Munday, Xuchen Zhang
William Munday, Department of Pathology, Yale University School of Medicine, New Haven, CT 06520, United States
Xuchen Zhang, Department of Pathology, VA Connecticut Health System and Yale University School of Medicine, West Haven, CT 06516, United States
Author contributions: Munday W performed review of the literature and drafted the manuscript; Zhang X provided overall intellectual input into the design and edited the final version of the manuscript; all authors approved the final version to be published.
Correspondence to: Xuchen Zhang, MD, PhD, Department of Pathology, VA Connecticut Health System and Yale University School of Medicine, 950 Campbell Ave. West Haven, CT 06516, United States. xuchen.zhang@yale.edu
Telephone: +1-203-9325711 Fax: +1-203-9374704
Received: January 21, 2014
Revised: March 26, 2014
Accepted: April 30, 2014
Published online: August 14, 2014
Abstract

Recent studies have suggested the existence of a patient population with esophageal eosinophilia that responds to proton pump inhibitor therapy. These patients are being referred to as having proton pump inhibitor responsive esophageal eosinophilia (PPI-REE), which is currently classified as a distinct and separate disease entity from both gastroesophageal reflux disease (GERD) and eosinophilic esophagitis (EoE). The therapeutic effect of proton pump inhibitor (PPI) on PPI-REE is thought to act directly at the level of the esophageal mucosa with an anti-inflammatory capacity, and completely independent of gastric acid suppression. The purpose of this manuscript is to review the mechanistic data of the proposed immune modulation/anti-inflammatory role of the PPI at the esophageal mucosa, and the existence of PPI-REE as a distinct disease entity from GERD and EoE.

Keywords: Gastroesophageal reflux disease, Eosinophilic esophagitis, Proton pump inhibitor responsive esophageal eosinophilia, Proton pump inhibitor

Core tip: The concept of pump inhibitor responsive esophageal eosinophilia (PPI-REE) is relatively new. The underlying mechanism(s) of PPI-REE pathogenesis and therapeutic effect of the PPI are still unknown. It is currently still unclear if PPI-REE is a subtype of gastroesophageal reflux disease (GERD), an eosinophilic esophagitis (EoE) phenotype, or a distinct entity. The aim of this manuscript is to review the mechanistic data of the proposed immune modulation/anti-inflammatory role of PPI at the esophageal mucosa, and the existence of PPI-REE as a distinct disease entity from GERD and EoE.