Newly developed and validated eosinophilic esophagitis histology scoring system and evidence that it outperforms peak eosinophil count for disease diagnosis and monitoring

Abstract

Summary Eosinophilic esophagitis (EoE) is diagnosed by symptoms, and at least 15 intraepithelial eosinophils per high power field in an esophageal biopsy. Other pathologic features have not been emphasized. We developed a histology scoring system for esophageal biopsies that evaluates eight features: eosinophil density, basal zone hyperplasia, eosinophil abscesses, eosinophil surface layering, dilated intercellular spaces (DIS), surface epithelial alteration, dyskeratotic epithelial cells, and lamina propria fibrosis. Severity (grade) and extent (stage) of abnormalities were scored using a 4-point scale (0 normal; 3 maximum change). Reliability was demonstrated by strong to moderate agreement among three pathologists who scored biopsies independently (P ≤ 0.008). Several features were often abnormal in 201 biopsies (101 distal, 100 proximal) from 104 subjects (34 untreated, 167 treated). Median grade and stage scores were significantly higher in untreated compared with treated subjects (P ≤ 0.0062). Grade scores for features independent of eosinophil counts were significantly higher in biopsies from untreated compared with treated subjects (basal zone hyperplasia P ≤ 0.024 and DIS P ≤ 0.005), and were strongly correlated (R-square >0.67). Principal components analysis identified three principal components that explained 78.2% of the variation in the features. In logistic regression models, two principal components more closely associated with treatment status than log distal peak eosinophil count (PEC) (R-square 17, area under the curve (AUC) 77.8 vs. R-square 9, AUC 69.8). In summary, the EoE histology scoring system provides a method to objectively assess histologic changes in the esophagus beyond eosinophil number. Importantly, it discriminates treated from untreated patients, uses features commonly found in such biopsies, and is utilizable by pathologists after minimal training. These data provide rationales and a method to evaluate esophageal biopsies for features in addition to PEC.

Histological Features of Eosinophilic Esophagitis in Children and Adolescents

“…Therefore, the histological diagnosis of EoE should not rely only on eosinophilic density. Indeed, a new histological score (the EoE Specific Histologic Scoring System) has been proposed for disease assessment, and it includes several variables beyond eosinophilic counts, such as basal zone hyperplasia, eosinophil abscesses, surface epithelial alteration, dyskeratosis, dilated intercellular spaces and fibrosis (4) . Only one patient presented H. pylori gastritis.…”

Cohort Profile: The Swiss Eosinophilic Esophagitis Cohort Study (SEECS)

“…Histologic activity has been evaluated by counting the peak eosinophil number per high-power field (400-fold magni-Inflamm Intest Dis 2017;2:163-170 DOI: 10.1159/000486131 fication). Recently, a histologic score has been published that contains 8 distinct items for the assessment of EoE activity [26]. In EoE, both PRO and ClinRO measures contribute to the assessment of disease activity [21].…”

La mystérieuse œsophagite à éosinophiles garde encore quelques secrets

“…Des approches non-invasives d'échantillonnage de la muqueuse oesophagienne prometteuses devraient permettre de moins recourir à l'endoscopie [77,78]. Parailleurs, de nouveaux scores d'activité clinique ou histologiques validésp ermettrontd esoutenir un développement clinique robuste [79,15]. Enfin, l'élucidation des mécanismes moléculaires entraînant l'EoE ouvre des voies prometteusesv ers de nouvelles approchest h é rapeutiques.…”

“…Ces analyses plus complètes permettent en particulier de valider des scores histologiques qui serviront à caractériser de manière plus précise chaque patient, dans le cadre du diagnostic mais aussi dans celui de l'évaluation de la réponse au traitement lors d'essais cliniques [15]. En fait, et nous reviendrons sur ce point en discutant de l'étiologied el 'EoE, une observation récente de l'équipe du professeur Straumann vient questionner l'importance même des éosinophiles dans l'EoE [16].…”