嗜酸性粒细胞性胃肠道疾病是一组罕见的疾病,其特征是嗜酸性粒细胞在胃肠道壁中的浸润量大于稳态条件。非食道嗜酸性粒细胞性胃肠道疾病是众所周知的嗜酸性粒细胞性食管炎以外的所有嗜酸性粒细胞性胃肠道疾病的总称。这包括嗜酸性粒细胞性胃炎,嗜酸性粒细胞性肠炎和嗜酸性粒细胞性结肠炎。这三种疾病的临床表现不典型,差异不大。浸润深度对表现症状的影响大于疾病位置。尽管随着时间的推移,该主题的诊断和研究频率正在增加,非食管嗜酸细胞性疾病很少见,目前高质量的证据有限.在这篇叙述性评论中,我们概述了病理生理学的最新见解,诊断方法和可用的治疗方案。转录组研究发现发病机制是2型T辅助细胞驱动的。各种实验室发现可用于引发怀疑和内窥镜检查检查。由于大多数情况下粘膜的内窥镜外观是正常的,需要在每个部分进行多次活检以量化组织中嗜酸性粒细胞的数量.用于诊断的嗜酸性粒细胞截止是一个有争议的话题,仍然缺乏共识。最近开发的基于组织的诊断平台,其测量差异表达的基因,将来可能可用于将具有中间嗜酸性组织水平的患者分类。为了治疗,皮质类固醇仍然是治疗的基石,但有希望的研究表明生物制剂的作用,特别是如Lirentelimab(反siglec8)。
Eosinophilic gastrointestinal disorders are a group of rare diseases characterized by the infiltration of eosinophils in the gastrointestinal wall in a greater amount than in homeostatic conditions. \'Non-esophageal eosinophilic gastrointestinal disorders\' is the umbrella term for all eosinophilic gastrointestinal disorders outside of the well known eosinophilic esophagitis. This includes eosinophilic gastritis, eosinophilic enteritis and eosinophilic colitis. The clinical presentation is atypical and not very different for the three disorders. The depth of infiltration has a bigger influence on the presenting symptoms than the disease location. Although the frequency of diagnosis and research in this subject is increasing over time, non-esophageal eosinophilic disorders are rare and high quality evidence is limited to date. In this narrative
review, we provide an overview of the latest insights in the pathophysiology, diagnostic approach and available treatment options. Transcriptome studies have found the pathogenesis to be T helper type 2 driven. Various laboratory findings can be used to trigger raised suspicion and investigation with endoscopy. As the endoscopic appearance of the mucosa is normal in most cases, multiple biopsies in each segment are needed to quantify the amount of eosinophils in the tissue. Eosinophilic cut-offs for diagnosis are a controversial topic and a consensus is still lacking. A recently developed tissue based diagnostic platform which measures differentially expressed genes might be available in the future to classify patients with intermediate eosinophilic tissue levels under the cut-off. For the treatment, corticosteroids are still the cornerstone of treatment but promising research suggests a role of biologicals, such as Lirentelimab (anti-siglec 8) in particular.