A Clinical Severity Index for Eosinophilic Esophagitis: Development, Consensus, and Future Directions

Abstract

Background & aims: Disease activity and severity of eosinophilic esophagitis (EoE) dictate therapeutic options and management, but the decision-making process for determining severity varies among practitioners. To reduce variability in practice patterns and help clinicians monitor the clinical course of the disease in an office setting, we aimed to create an international consensus severity scoring index for EoE.

Methods: A multidisciplinary international group of adult and pediatric EoE researchers and clinicians, as well as non-EoE allergy immunology and gastroenterology experts, formed 3 teams to review the existing literature on histology, endoscopy, and symptoms of EoE in the context of progression and severity. A steering committee convened a 1-day virtual meeting to reach consensus on each team's opinion on salient features of severity across key clinicopathologic domains and distill features that would allow providers to categorize disease severity.

Results: Symptom features and complications and inflammatory and fibrostenotic features on both endoscopic and histologic examination were collated into a simplified scoring system-the Index of Severity for Eosinophilic Esophagitis (I-SEE)-that can be completed at routine clinic visits to assess disease severity using a point scale of 0-6 for mild, 7-14 for moderate, and ≥15 for severe EoE.

Conclusions: A multidisciplinary team of experts iteratively created a clinically usable EoE severity scoring system denominated "I-SEE" to guide practitioners in EoE management by standardizing disease components reflecting disease severity beyond eosinophil counts. I-SEE should be validated and refined using data from future clinical trials and routine clinical practice to increase its utilization and functionality.

Keywords: Complications; Endoscopy; Eosinophilic Esophagitis; Histology; Severity; Symptoms.

FIGURE 1.

Inflammatory Endoscopic Findings. Examples of localized findings include (A) focal exudates in the proximal esophaugs, (B) exudates, furrows, and edema in the distal esophagus, and (C) exudates, furrows, and edema in the mid esophagus. Examples of diffuse findings throughout the esophagus include (D) exudates, furrows, and edema, (E) marked exudates and edema, and (F) exudates and edema (also with the presence of rings).

FIGURE 2.

Representative pathologic findings of EoE pertinent for the severity index. The findings in panels A, B, and C can best be interpreted for use in the I-SEE histology sections by answering the following series of questions: Question 1: Is basal zone hyperplasia exceeding one-third of total epithelial thickness (bar in Panel A) present? ___Yes ___No Question 2: Is lamina propria present and not crushed (arrows in Panel B)? ___Yes, go to question 3 and do not complete questions 4 and 5 ___No, do not complete question 3 and go to questions 4 and 5 Question 3: Is any lamina propria fibrosis (arrows in Panel B) present? ___Yes ___No Question 4: Is surface epithelial alteration (arrow in Panel A), with or without admixed eosinophils, attached or partially/completely detached, present? ___Yes ___No Question 5: Are dyskeratotic epithelial cells (arrow in Panel C?) present? ___Yes ___No

FIGURE 3.

Fibrotic Endoscopic Findings. Examples include (A) mild rings where the endoscope passes easily, (B) prominent rings with some narrowing where dilation is required and the endoscope passes with a “snug fit”, and (C) severe rings with a focal stricture that precludes passage of a standard upper endoscope.