Esophageal Fibrosis in Eosinophilic Gastrointestinal Diseases

Abstract

Objectives: Eosinophilic esophagitis (EoE), the most common eosinophilic gastrointestinal disease (EGID), is associated with lamina propria (LP) fibrosis. The relationship of EoE to other EGIDs is still unclear. We frequently observe cases of concurrent esophageal eosinophilia and extra-esophageal mucosal eosinophilia. The purpose of this study was to compare clinical, endoscopic, and histologic features, as well as the prevalence of esophageal LP fibrosis in children with EGID and concurrent esophageal eosinophilia to children with EoE. We also examine the current practices of pathologists in evaluating fibrosis. Methods: We reviewed esophageal biopsies from index cases of EoE (N = 38), EGID with significant esophageal eosinophilia (≥15 eos/hpf) (EGID-SEE, N = 38), EGID with mild esophageal eosinophilia (1–14 eos/hpf) (EGID-MEE, N = 12), and EGID with no esophageal eosinophilia (EGID-NEE, N = 12) for LP presence, adequacy, and fibrosis. Results: EoE and EGID-SEE cases share similar demographics, esophageal endoscopic features, and symptoms. A majority of EGID-SEE cases (71%) had adequate LP for the evaluation of fibrosis, similar to EoE cases (87%). The prevalence of esophageal fibrosis in EoE (79%) and EGID-SEE (55%) cases were similar, whereas no fibrosis was detected in the EGID-MEE and EGID-NEE cases. The fibrosis was patchy and often detected in the distal esophagus. Fourteen cases were reclassified from their original clinical diagnosis as having fibrosis by the study pathologists. Conclusions: Cases of EGID-SEE have overlapping features with EoE, suggesting that all EGIDs are part of a disease continuum. A consensus for the evaluation of LP fibrosis is needed.

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.…”

Challenges in Inter-rater Agreement on Lamina Propria Fibrosis in Esophageal Biopsies

“…6 A subsequent study at our institution confirmed that LP fibrosis not only occurs in EoE, but also in patients with eosinophilic gastrointestinal diseases (EGIDs) with significant esophageal eosinophilia. 7…”

“…6 A subsequent study at our institution confirmed that LP fibrosis not only occurs in EoE, but also in patients with eosinophilic gastrointestinal diseases (EGIDs) with significant esophageal eosinophilia. 7 Currently, there is no standard histologic definition of lamina propria fibrosis or its adequacy. Hence, many pathologists do not routinely include such features in their reports.…”