{"title":"Real-world efficacy of dupilumab in four cases of paediatric-onset fibrostenotic eosinophilic esophagitis","authors":"Sophia A. Patel","doi":"10.1111/1440-1681.13903","DOIUrl":null,"url":null,"abstract":"<p>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.</p>","PeriodicalId":50684,"journal":{"name":"Clinical and Experimental Pharmacology and Physiology","volume":null,"pages":null},"PeriodicalIF":2.9000,"publicationDate":"2024-06-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Clinical and Experimental Pharmacology and Physiology","FirstCategoryId":"3","ListUrlMain":"https://onlinelibrary.wiley.com/doi/10.1111/1440-1681.13903","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 0
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.
期刊介绍:
Clinical and Experimental Pharmacology and Physiology is an international journal founded in 1974 by Mike Rand, Austin Doyle, John Coghlan and Paul Korner. Our focus is new frontiers in physiology and pharmacology, emphasizing the translation of basic research to clinical practice. We publish original articles, invited reviews and our exciting, cutting-edge Frontiers-in-Research series’.