INTRODUCTION Ulcerative jejunitis (UJ) or ulcerative enteritis (UE) is a rare complication of celiac disease (CeD). Guidelines regarding diagnosis and management are missing and these cases have seldom been reported in the United States. DESIGN Single center case-series of CeD in which UE developed at a large academic center in the USA. Clinical presentation, diagnosis, treatment, and evolution of disease were collected. RESULTS Eight cases were identified (6M/2F, mean age 59.5 (38-77) years). Presentations included intestinal obstruction (n=3), GI hemorrhage (n=3), and malabsorption (n=2). Ulcers were present in the duodenum in 4 patients, and exclusively past the angle of Treitz in only 4 cases, which makes the term ulcerative enteritis (UE) more appropriate than UJ. Six out of eight had T-cell receptor (TCR) clonal gene rearrangements and two had definite aberrant T cells. Corticosteroids were tried in all patients without improvement and 5 underwent surgical resection. Three patients received cladribine. One patient received an autologous stem cell transplant, followed by ruxolitinib. Two were subsequently diagnosed with enteropathy-associated T-cell lymphoma (EATL), including one with cerebral EATL, and 1 died from hemophagocytic syndrome. Two are still alive, including one only on GFD and two were lost to follow-up after surviving at least 30 months post treatment. CONCLUSION UE seems a more appropriate term to describe an ulcerative complication of CeD at high risk of obstruction or bleeding. Steroids were not effective. Treatment outcomes were variable, but with a 50% death rate.

中文翻译：

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乳糜泻中的溃疡性空肠炎：三十年的美国经验。


引言 溃疡性空肠炎 （UJ） 或溃疡性肠炎 （UE） 是乳糜泻 （CeD） 的一种罕见并发症。缺乏有关诊断和管理的指南，这些病例在美国很少报告。设计 UE 在美国一家大型学术中心开发的 CeD 单中心案例系列。收集了临床表现、诊断、治疗和疾病进展。结果 确定了 8 例 （6M/2F，平均年龄 59.5 （38-77） 岁）。表现包括肠梗阻 （n=3） 、胃肠道出血 （n=3） 和吸收不良 （n=2）。4 例患者十二指肠存在溃疡，只有 4 例完全超过了 Treitz 角，这使得溃疡性肠炎 （UE） 一词比 UJ 更合适。8 例中有 6 例具有 T 细胞受体 （TCR） 克隆基因重排，2 例具有明确的异常 T 细胞。所有患者均尝试使用皮质类固醇，但无改善，5 例接受了手术切除。3 例患者接受了克拉屈滨治疗。1 例患者接受了自体干细胞移植，随后接受了 ruxolitinib。2 例随后被诊断为肠病相关 T 细胞淋巴瘤 （EATL），包括 1 例脑 EATL，1 例死于噬血细胞综合征。2 例仍然存活，包括 1 例仅在 GFD 上，2 例在治疗后存活至少 30 个月后失访。结论 UE 似乎更适合描述梗阻或出血风险高的 CeD 溃疡并发症。类固醇无效。治疗结果各不相同，但死亡率为 50%。