Real-world efficacy of dupilumab in four cases of paediatric-onset fibrostenotic eosinophilic esophagitis

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. Eosinophilic esophagitis is a chronic, progressive disease. Over time, it can lead to inflammation, followed by fibrosis. Fibrosis is mediated by unchecked inflammation, and possibly also associated with a specific phenotype of Eosinophilic Esophagitis. Patients in our study who were treated with dupilumab with history of fibrosis defined by endoscopic and visual findings such as strictures, rings, and narrowing or requiring dilation, demonstrate excellent resolution of fibrosis symptoms following treatment with dupilumab. image