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

IF 2.9 4区 医学 Q2 Medicine
Sophia A. Patel
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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.

Abstract Image

杜匹单抗对四例儿科纤维性嗜酸性粒细胞食管炎的实际疗效。
嗜酸性粒细胞食管炎(EoE)是一种日益流行的免疫介导疾病,会导致食管发生慢性变化。这些变化包括狭窄、狭窄和狭窄,由白细胞介素(IL)-13 途径介导,通过增加成纤维细胞的迁移和胶原沉积 1 导致上皮下纤维化,从而导致重塑和纤维化。IL-13可下调TSPAN12基因,该基因的表达可调节纤维化,并导致屏障功能的改变和咽喉炎患者纤维增生率的升高。杜比鲁单抗是一种旨在阻断 IL-13 的生物疗法,在临床试验中已被证明能改善与咽喉炎相关的炎症和纤维化。我们在此报告了四例有记录的食道狭窄患者,他们因结构性疾病而无法通过儿科内窥镜,需要进行扩张术。
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来源期刊
CiteScore
6.20
自引率
0.00%
发文量
128
审稿时长
6 months
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’.
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