Digestive Diseases and Sciences

, Volume 57, Issue 5, pp 1413–1419 | Cite as

Transient PPI Responsive Esophageal Eosinophilia May Be a Clinical Sub-phenotype of Pediatric Eosinophilic Esophagitis

Case Report

Abstract

Background

Eosinophilic esophagitis (EoE) and gastroesophageal reflux (GERD) both cause esophageal eosinophilia. Reports show that esophageal eosinophilia meeting criteria for EoE may respond to acid suppression mono-therapy. Consensus guidelines have termed this entity “PPI-responsive esophageal eosinophilia” (PPIRee) and recommend a trial with proton-pump inhibitors (PPIs) prior to a definitive EoE diagnosis. The mechanisms of PPIRee and whether this represents a sub-phenotype of GERD, a sub-phenotype of EoE, or its own distinct entity remain unclear.

Methods

A database search revealed children who had an initial histologic response to PPI monotherapy but had recurrence of esophageal eosinophilia and symptoms despite continued PPI therapy. In order to understand the patterns of esophageal inflammatory cells during PPI therapy we performed quantitative immunohistochemistry for mast cells, CD1a positive antigen presenting cells, and CD45RO memory T cells.

Results

Four pediatric patients (mean age 9.5 years) had a mean peak eosinophil count of 52 eos/hpf which initially resolved completely during PPI mono-therapy. However, despite continued PPI therapy, endoscopic abnormalities and pan-esophageal eosinophilia recurred (mean peak eosinophil count of 64 eos/hpf). There was no seasonal variation or lack of PPI adherence that explained the return of eosinopihlia. Similar to eosinophilia, mastocytosis and CD45RO cells were transiently decreased during PPI therapy.

Conclusion

PPIs appear to be capable of transiently resolving multiple inflammatory cell subsets including eosinophils, mast cells, and CD45RO cells. Our data suggest that patients with PPIRee should have continued monitoring for EoE during PPI monotherapy. The numbers of patients in whom PPIRee is a transient phenomenon and whether PPIRee represents a sub-phenotype of EoE in children merits further investigation.

Keywords

Proton-pump inhibitors Mast cells CD45RO cells 

Notes

Acknowledgments

The authors would like to thank Kevin Alford for his contributions in the laboratory and also Dr.’s Ikuo Hirano and Glen Furuta for their comments. Support was provided by a grant from The Hearst Foundation.

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Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • Ranjan Dohil
    • 1
    • 2
  • Robert O. Newbury
    • 1
    • 2
  • Seema Aceves
    • 1
    • 2
  1. 1.Departments of Pediatrics and PathologyUniversity of California, San DiegoLa JollaUSA
  2. 2.Rady’s Children’s HospitalSan DiegoUSA

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