Clinical manifestations,diagnosis and long-term prognosis of adult autoimmune enteropathy:Experience from Peking Union Medical College Hospital
作者机构：Department of GastroenterologyChinese Academy of Medical Sciences&Peking Union Medical College HospitalBeijing 100730China Department of PathologyChinese Academy of Medical Scinences&Peking Union Medical College HospitalBeijing 100730China Department of PathologyTianjin First Central HospitalNankai UniversityTianjin 300192China Affairs OfficeChinese Academy of Medical Sciences&Peking Union Medical College Hospital(West campus)Beijing 100032China
出 版 物：《World Journal of Gastroenterology》 (世界胃肠病学杂志（英文版）)
年 卷 期：2024年第30卷第19期
页 面：2523-2537页
核心收录：
学科分类：1004[医学-公共卫生与预防医学(可授医学、理学学位)] 1002[医学-临床医学] 100401[医学-流行病与卫生统计学] 10[医学]
基 金：Supported by National High Level Hospital Clinical Research Funding,No.2022-PUMCH-B-022 and No.2022-PUMCH-D-002 CAMS Innovation Fund for Medical Sciences,No.2021-1-I2M-003 Undergraduate Innovation Program,No.2023-zglc-06034 National Key Clinical Specialty Construction Project,No.ZK108000
主 题：Autoimmune enteropathy Clinical manifestations Diagnostic criteria Pathological features Long-term prognosis
摘 要：BACKGROUND Autoimmune enteropathy(AIE)is a rare disease whose diagnosis and long-term prognosis remain challenging,especially for adult AIE *** To improve overall understanding of this disease’s diagnosis and *** We retrospectively analyzed the clinical,endoscopic and histopathological characteristics and prognoses of 16 adult AIE patients in our tertiary medical center between 2011 and 2023,whose diagnosis was based on the 2007 diagnostic *** Diarrhea in AIE patients was characterized by secretory *** common endoscopic manifestations were edema,villous blunting and mucosal hyperemia in the duodenum and *** blunting(100%),deep crypt lymphocytic infiltration(67%),apoptotic bodies(50%),and mild intraepithelial lymphocytosis(69%)were observed in the duodenal ***,there were other remarkable abnormalities,including reduced or absent goblet cells(duodenum 94%,ileum 62%),reduced or absent Paneth cells(duodenum 94%,ileum 69%)and neutrophil infiltration(duodenum 100%,ileum 69%).Our patients also fulfilled the 2018 diagnostic criteria but did not match the 2022 diagnostic criteria due to undetectable anti-enterocyte *** patients received glucocorticoid therapy as the initial medication,of which 14/16 patients achieved a clinical response in 5(IQR:3-20)*** were administered to 9 patients with indications of steroid dependence(6/9),steroid refractory status(2/9),or intensified maintenance medication(1/9).During the median of 20.5 months of followup,2 patients died from multiple organ failure,and 1 was diagnosed with non-Hodgkin’s *** cumulative relapse-free survival rates were 62.5%,55.6%and 37.0%at 6 months,12 months and 48 months,*** Certain histopathological findings,including a decrease or disappearance of goblet and Paneth cells in intestinal biopsies,might be potential diagnostic criteria for adult *** long-term prognosis is still unsatisfac
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