ABSTRACT
Eosinophilic esophagitis (EoE) is an increasingly prevalent immune-mediated disease that leads to chronic changes in the oesophagus. These changes can include strictures, narrowing, and stenosis, mediated by an interleukin (IL)-13 pathway, which leads to remodelling and fibrosis through increasing migration of fibroblasts and subepithelial fibrosis via collagen deposition 1. IL-13 downregulates TSPAN12, a gene whose expression regulates fibrosis and causes changes in barrier function and higher rates of fibrostenosis in EoE. Dupilumab, a biologic therapy aimed at blocking IL-13, has been shown to improve EoE-related inflammation and fibrosis in clinical trials. We report here four unique patients with documented oesophageal stenosis with inability to pass a paediatric endoscope due to structuring disease, requiring dilation, who had resolution of their oesophageal narrowing following dupilumab therapy.
Subject(s)
Antibodies, Monoclonal, Humanized , Eosinophilic Esophagitis , Humans , Eosinophilic Esophagitis/drug therapy , Eosinophilic Esophagitis/pathology , Antibodies, Monoclonal, Humanized/therapeutic use , Antibodies, Monoclonal, Humanized/pharmacology , Male , Female , Child , Fibrosis , Treatment Outcome , Esophageal Stenosis/drug therapy , Esophageal Stenosis/pathology , Child, Preschool , AdolescentABSTRACT
Eosinophilic esophagitis is a chronic, immune-mediated esophageal condition that may lead to impairment of quality of life in pediatric and young adult patients. We performed a prospective, cross-sectional study on 40 patients between the ages of 2-21 years with an established diagnosis of eosinophilic esophagitis. The study evaluated physical, emotional, social, and school functioning in patients undergoing treatment with proton pump inhibitors, dietary elimination, or swallowed corticosteroids. There were no statistically significant differences in total or domain-specific quality of life scores between proton pump inhibitors, dietary elimination, and swallowed corticosteroid therapy. Overall, total and domain-specific quality of life were well-preserved in patients with eosinophilic esophagitis, with the highest scores reported in social functioning. There were also no statistically significant associations between clinical, endoscopic, and histologic features and quality-of-life measures.