A new eosinophilic esophagitis (EoE)-like disease without tissue eosinophilia found in EoE Families

Straumann, A; Blanchard, C; Radonjic-Hoesli, Susanne; Bussmann, Ch; Hruz, P; Safroneeva, Ekaterina; Simon, Dagmar; Schoepfer, A M; Simon, Hans-Uwe (2016). A new eosinophilic esophagitis (EoE)-like disease without tissue eosinophilia found in EoE Families. Allergy, 71(6), pp. 889-900. Wiley-Blackwell 10.1111/all.12879

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BACKGROUND Eosinophilic esophagitis (EoE) is a rapidly emerging, chronic inflammatory, genetically impacted disease of the esophagus, defined clinically by symptoms of esophageal dysfunction and, pathologically, by an eosinophil-predominant tissue infiltration. However, in four EoE-families, we have identified patients presenting with EoE-typical and corticosteroid-responsive symptoms, but without tissue eosinophilia. It was the aim of this study to clinically and immunologically characterize these patients with EoE-like disease. METHODS Five patients suffering from an EoE-like disease were evaluated with endoscopic, histologic, functional and quantitative immunohistologic examinations, and mRNA expression determination. RESULTS The frequency of first generation offspring of EoE-like disease patients affected by EoE or EoE-like disease was 40%. Immunofluorescence analysis confirmed an almost complete absence of eosinophils in the esophageal tissues of patients with EoE-like disease, but revealed a considerable T cell infiltration, comparable to EoE. In contrast to EoE, eotaxin-3 mRNA and protein were markedly reduced in EoE-like disease (P < 0.05). The mRNA expression levels of three selected EoE genes (eotaxin-3, MUC4 and CDH26) allowed to discriminate between EoE-like disease, EoE and normal epithelium. CONCLUSIONS Patients suffering from "EoE without eosinophilia" do not fulfill formally the diagnostic criteria for EoE. However, their clinical manifestation, immunohistology and gene-expression pattern, plus the fact that they bequeath EoE to their offspring, suggest a uniform underlying pathogenesis. Conventional EoE, with its prominent eosinophilia, therefore appears to be only one phenotype of a broader "inflammatory dysphagia syndrome" spectrum. In this light, the role of the eosinophils, the definition of EoE, and its diagnostic criteria must likely be reconsidered. This article is protected by copyright. All rights reserved.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Dermatology
04 Faculty of Medicine > Pre-clinic Human Medicine > Institute of Pharmacology
04 Faculty of Medicine > Pre-clinic Human Medicine > Institute of Social and Preventive Medicine

UniBE Contributor:

Radonjic, Susanne Irene; Safroneeva, Ekaterina; Simon, Dagmar and Simon, Hans-Uwe

Subjects:

600 Technology > 610 Medicine & health
300 Social sciences, sociology & anthropology > 360 Social problems & social services

ISSN:

0105-4538

Publisher:

Wiley-Blackwell

Language:

English

Submitter:

Debora Scherrer

Date Deposited:

12 Jul 2016 11:21

Last Modified:

08 Sep 2017 16:33

Publisher DOI:

10.1111/all.12879

PubMed ID:

26970242

Uncontrolled Keywords:

dysphagia; eotaxin; esophagus; inflammation

BORIS DOI:

10.7892/boris.80202

URI:

https://boris.unibe.ch/id/eprint/80202

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