Subtyping of Eosinophilic Esophagitis Based on Disease Presentation in a pediatric Cohort

Abstract

Objective: Eosinophilic esophagitis (EoE) is an immune-mediated inflammatory disease characterized by eosinophilic infiltration of esophageal tissue. Subtyping of EoE patients could be useful in predicting therapeutic response. We propose clinical subtypes, apply them to our pediatric EoE population retrospectively, and assess therapy choices and remission at one year. Methods: A retrospective chart review of pediatric patients diagnosed with EoE was conducted. Patients were grouped into proposed subtypes (severe, allergic, fibrostenotic, inflammatory, unclassified) based on presenting characteristics. The primary outcome was histologic remission, which was defined <15 eosinophils/high-powered-field (hpf) at the closest visit 1 year postdiagnosis. Results: Subtyping was possible in 242 of 256 patients and follow-up histological data were available in 75 subjects. The majority had an overlap in phenotype with 17% severe, 77% allergic, 15% fibrostenotic, 60% inflammatory, and 5% unclassified, whereas 45% of the cohort were assigned to a unique subtype. At 1 year, 43/75 (57%) of patients achieved histologic remission, with an overall average decrease of 33 (IQR −47, −12) eosinophils/hpf across the entire cohort. There was no difference in remission rates among subtypes. First-line therapy review revealed higher rates of proton pump inhibitor (PPI) ± topical steroids utilization in severe patients, while topical steroids were prescribed preferentially over dietary therapy in the fibrostenotic subtype. Conclusion: There were no observed differences in remission rates at 1 year among clinically defined subtypes of EoE, although this could be attributed to overlapping subtypes. Most patients responded well to medical therapy. Larger scale prospective studies designed to subtype patients and protocolize treatment may help personalize the approach to EoE management.

Allergic phenotype identified on allergen testing is associated with proton pump inhibitor nonresponse in eosinophilic esophagitis

“…Indeed, some previous data have suggested that once a certain threshold of Th2‐mediated inflammation is surpassed, there may be a drop in PPI responsiveness 14 . Another consideration is that an allergic phenotype in EoE may indicate more severe disease, and prior pediatric studies have suggested that those with PPI‐r‐EoE tend to have mild to moderate disease 24,25 . Finally, reflux and refluxate‐induced mucosal injury may play a less prominent role in EoE patients with allergic phenotype, further reducing the impact of PPI therapy.…”

Challenging assumptions about the demographics of eosinophilic gastrointestinal diseases: A systematic review

“… 42 , 43 Ascertaining the true demographics of EGIDs also helps us understand the heterogeneity of these diseases through identification of various disease phenotypes. As phenotypes are recognized, we can explore genotypic and possible mechanistic differences 17 , 44 and tailor therapies for better outcomes. On a larger scale, understanding disease demographics is crucial for resource allocation for clinical care, research, community engagement, and population health.…”

Reproducibility and Generalizability of the Web-Based Tool to Predict Lamina Propria Fibrosis in Eosinophilic Esophagitis

“…Future studies are warranted to further test the reproducibility and generalizability of the web‐based predictive models in different populations to make it more robust and reliable, as well as to update the existing web‐based predictive models to provide predictive probability for severity of LPF by sub‐scores (0–3) per EoEHSS. It is conceivable that we might be able to create models to prospectively predict LPF in different EoE phenotypes (17) and consistent evaluation of LPF (18) in EoE patients with high degree of confidence.…”