Eosinophils Beyond the Esophagus: A Review of Non-EoE Eosinophilic Gastrointestinal Diseases.

For eosinophilic esophagitis (EoE), the most well researched of the eosinophilic gastrointestinal diseases (EGIDs), there is a plethora of knowledge for its diagnosis and management; however, much less guidance is available for the non-EoE EGIDs. Efforts have been made to characterize the clinical features, epidemiology, diagnosis, and natural history of EGIDs, as the frequency of the non-EoE EGIDs has continued to rise. The diagnosis of the different non-EoE EGIDs, eosinophilic gastritis, enteritis, and colitis, can be challenging because of their rarity and heterogeneous presentations which can lead to delayed diagnosis and poor health-related quality of life in affected patients.

Initial Validation of the Eosinophilic Gastritis and Gastroenteritis Quality of Life Scale.

Background And Aims: Eosinophilic gastrointestinal disorders (EGIDs) are chronic immune-mediated conditions characterized by gastrointestinal symptoms and gut eosinophilia. Health-related quality of life (HRQoL) is an important metric to understand patient outcomes; the only disease-specific measure of HRQoL exists for eosinophilic esophagitis (EoE) patients. Patients with non-EoE EGIDs experience unique symptoms and treatment options and disease burden of these rare disorders is under-recognized and considered significant.

Systematic Review: Variability in Definitions of Fibrostenosis in Eosinophilic Oesophagitis.

Background: Fibrostenosis is a serious complication of eosinophilic oesophagitis, but there is a lack of consensus regarding its definition and assessment. This poses a barrier in clinical care and research.

Aim: To perform a systematic review to examine existing definitions and diagnostic methods of detection regarding fibrostenosis in eosinophilic oesophagitis.

Esophageal epithelial Ikkβ deletion promotes eosinophilic esophagitis in experimental allergy mouse model.

Background: Eosinophilic esophagitis (EoE) is a chronic T2-associated inflammatory disorder triggered by food allergens, resulting in esophageal dysfunction through edema, fibrosis, and tissue remodeling. The role of epithelial remodeling in EoE pathogenesis is critical but not fully understood.

Objective: We investigated the role of epithelial IKKβ/NF-κB signaling in EoE pathogenesis using a mouse model with conditional Ikkβ knockout in esophageal epithelial cells (Ikkβ).

The Minimally Invasive 1-Hour Esophageal String Test Monitors Therapeutic Changes in Mucosal Inflammation in Eosinophilic Esophagitis.

Introduction: Endoscopy, standard-of-care for monitoring eosinophilic esophagitis (EoE), assesses mucosal inflammation. The Esophageal String Test (EST), a minimally invasive swallowed capsule and immunoassays, quantifies EoE inflammation. We determined whether the EST/EoEScore can monitor disease in patients undergoing treatment.

Esophageal epithelial deletion promotes eosinophilic esophagitis in experimental allergy mouse model.

Background: Eosinophilic esophagitis (EoE) is a chronic T helper type 2 (Th2)-associated inflammatory disorder triggered by food allergens, resulting in esophageal dysfunction through edema, fibrosis, and tissue remodeling. The role of epithelial remodeling in EoE pathogenesis is critical but not fully understood.

Objective: To investigate the role of epithelial IKKβ/NFκB signaling in EoE pathogenesis using a mouse model with conditional β knockout in esophageal epithelial cells ( β ).

Effect of benralizumab on histopathology and inflammatory signatures in a clinical cohort of eosinophilic esophagitis.

A preliminary report from the recent phase 3 trial of benralizumab, a monoclonal antibody that binds to interleukin-5 receptor alpha (IL5Rα), in patients with EoE revealed that medication use led to tissue eosinophil eradication but did not meet the clinical endpoint of symptom resolution. Here, we characterized the clinical, endoscopic, histologic, and transcriptional changes in patients with active EoE following benralizumab treatment. We retrospectively examined patients with EoE treated with benralizumab at the University of Utah (n = 11) and reviewed reported clinical symptoms, circulating and tissue eosinophilia, and endoscopic and histologic scores.

Nonepithelial Gene Expression Correlates With Symptom Severity in Adults With Eosinophilic Esophagitis.

Background: The mechanistic basis of the variable symptomatology seen in eosinophilic esophagitis (EoE) remains poorly understood.

Objective: We examined the correlation of a validated, patient-reported outcome metric with a broad spectrum of esophageal transcripts to uncover potential symptom pathogenesis.

Methods: We extracted data from 146 adults with EoE through the Consortium of Eosinophilic Gastrointestinal Disease Researchers.

Clinical and molecular correlates of the Index of Severity for Eosinophilic Esophagitis.

Background: The Index of Severity for Eosinophilic Esophagitis (I-SEE) is a new expert-defined clinical tool that classifies disease severity of eosinophilic esophagitis (EoE).

Objective: We aimed to determine whether I-SEE is associated with patient characteristics, molecular features of EoE, or both.

Methods: We analyzed a prospective cohort of patients with EoE from the Consortium of Eosinophilic Gastrointestinal Disease Researchers (CEGIR).

Dietary Management of Eosinophilic Esophagitis.

Eosinophilic esophagitis (EoE) is a chronic immune-mediated food allergy-driven disease characterized by eosinophilic inflammation of the esophagus leading to symptoms of esophageal dysfunction. Prior studies have supported the key role of food allergen exposure as the main driver behind the etiopathogenesis showing that removal of food antigens can result in disease remission in both children and adults. These landmark studies serve as the basis for the rising interest and evolution of dietary therapy in EoE.

Dietary Management of Eosinophilic Esophagitis.

Eosinophilic esophagitis (EoE) is a chronic immune-mediated food antigen-driven disease characterized by tissue eosinophilia and clinical symptoms of esophageal dysfunction. Medical and dietary therapies can be offered as treatment options in both pediatric and adult populations. Advances in nutritional research in EoE have produced different levels of dietary restriction, ranging from elimination of a single food group to more extensive restriction such as the two-food elimination diet, four-food elimination diet, or six-food elimination diet.

A Multicenter Long-Term Cohort Study of Eosinophilic Esophagitis Variants and Their Progression to Eosinophilic Esophagitis Over Time.

Introduction: Eosinophilic esophagitis (EoE) variants have been recently characterized as conditions with symptoms of esophageal dysfunction resembling EoE, but absence of significant esophageal eosinophilia. Their disease course and severity have yet to be determined.

Methods: Patients from 6 EoE centers with symptoms of esophageal dysfunction, but peak eosinophil counts of <15/hpf in esophageal biopsies and absence of gastroesophageal reflux disease with at least one follow-up visit were included.

Joint ESPGHAN/NASPGHAN Guidelines on Childhood Eosinophilic Gastrointestinal Disorders Beyond Eosinophilic Esophagitis.

Introduction: Eosinophilic gastrointestinal disorders beyond eosinophilic esophagitis (non-EoE EGIDs) are rare chronic inflammatory disorders of the gastrointestinal (GI) tract. Diagnosis is based on clinical symptoms and histologic findings of eosinophilic inflammation after exclusion of a secondary cause or systemic disease. Currently, no guidelines exist for the evaluation of non-EoE EGIDs.

Composite score of physiomechanical esophageal function using functional lumen imaging probe panometry in eosinophilic esophagitis.

Background And Aims: The evaluation provided by functional lumen imaging probe (FLIP) panometry includes esophageal distensibility/compliance (mechanics) of the esophageal body and esophagogastric junction (EGJ) and esophageal motility (secondary peristalsis). We developed a composite score using these parameters to characterize physiomechanical function in patients with eosinophilic esophagitis (EoE).

Methods: Two hundred fifteen adult patients with EoE who completed FLIP panometry during sedated endoscopy with esophageal biopsy sampling were included.

Suprabasal cells retain progenitor cell identity programs in eosinophilic esophagitis-driven basal cell hyperplasia.

Eosinophilic esophagitis (EoE) is an esophageal immune-mediated disease characterized by eosinophilic inflammation and epithelial remodeling, including basal cell hyperplasia (BCH). Although BCH is known to correlate with disease severity and with persistent symptoms in patients in histological remission, the molecular processes driving BCH remain poorly defined. Here, we demonstrate that BCH is predominantly characterized by an expansion of nonproliferative suprabasal cells that are still committed to early differentiation.

Prospective study of an amino acid-based elemental diet in an eosinophilic gastritis and gastroenteritis nutrition trial.

Background: Eosinophilic gastritis/gastroenteritis (EoG/EoGE) are rare disorders with pathologic gastric and/or small intestinal eosinophilia lacking an approved therapy. An allergic mechanism is postulated but underexplored mechanistically and therapeutically.

Objective: We evaluated the effectiveness of a food allergen-free diet (elemental formula) in controlling gastrointestinal eosinophilia in adult EoG/EoGE.

A PhysioMechanical Model of Esophageal Function in Eosinophilic Esophagitis.

Background & Aims: Eosinophilic esophagitis (EoE) is characterized by eosinophilic inflammation, but also heterogeneous presentations involving fibrostenotic esophageal remodeling and esophageal dysmotility. We aimed to define and evaluate phenotypes of EoE using functional lumen imaging probe (FLIP) panometry (ie, a PhysioMechanical classification of EoE).

Methods: Patients with EoE who completed FLIP during endoscopy were included in a cross-sectional study.

Bulk T-cell receptor sequencing confirms clonality in pediatric eosinophilic esophagitis and identifies a food-specific repertoire.

Background: Eosinophilic esophagitis (EoE) involves a chronic immune-mediated response to dietary antigens. Recent work identifies T-cell clonality in children with EoE, however, it is unknown whether this is true in adults or whether there is a restricted food-specific T-cell repertoire. We sought to confirm T-cell receptor (TCR) clonality in EoE and assess for differences with specific food triggers.