Adult-onset autoimmune enteropathy in the setting of thymoma successfully treated with infliximab
Adult-onset autoimmune enteropathy in the setting of thymoma successfully treated with infliximab
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DOI：
10.1007/s10620-005-2666-x
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发表时间：
2005-05-01
影响因子：
3.1
通讯作者：
Clouse, RE
中科院分区：
文献类型：
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作者：
Elwing, JE;Clouse, RE
自身免疫性肠病（AIE）的特点是慢性顽固性腹泻、吸收不良引起的体重减轻以及免疫介导的肠粘膜损伤。该综合征最初于 1982 年在一名男孩中被描述 (1, 2)。沃克-史密斯制定了一套仍在使用的疾病诊断标准：(a) 小肠绒毛萎缩，(b) 对饮食限制无反应，以及 (c) 循环肠细胞抗体和/或相关自身免疫性疾病 (3)。 AIE 也会发生在免疫球蛋白缺乏的情况下，免疫球蛋白缺乏是发生自身免疫性疾病的已知危险因素 (4, 5)。第一个发表的成人发病 AIE 病例出现在 1997 年，当时 Corazza 等人。描述了两名年龄分别为 38 岁和 47 岁的受试者，患有严重腹泻和吸收不良、小肠绒毛萎缩、对无麸质饮食无反应以及循环抗肠细胞抗体 (6, 7)。此后，仅出现了偶尔出现的成人患有这种疾病的病例报告，与儿科病例类似，成人发病的 AIE 与自身免疫和免疫球蛋白缺乏有关。 AIE 可能只是胃肠道系统更弥漫性自身免疫性疾病的一种表现，具有异质性表现，包括胃炎、结肠炎、肝炎和胰腺炎，以及多种自身抗体，例如抗壁细胞抗体和抗杯状细胞抗体 (4,8,9)。莱昂等人。最近创造了广义自身免疫性肠道疾病（GAGD）这个术语，用于指那些自身免疫性胃肠道损伤超出小肠的患者，
Autoimmune enteropathy (AIE) is characterized by chronic intractable diarrhea, weight loss from malabsorption, and immune-mediated damage to the intestinal mucosa. The syndrome initially was described in 1982 in a male child (1, 2). Walker-Smith formulated a set of diagnostic criteria for the disorder that remain in use:(a) small intestinal villous atrophy,(b) unresponsiveness to dietary restriction, and (c) circulating enterocyte antibodies and/or associated autoimmune conditions (3). AIE also occurs in the setting of immunoglobulin deficiencies, a known risk factor for the development of autoimmune disorders (4, 5).The first published cases of adult-onset AIE appeared in 1997 when Corazza et al. described two subjects aged 38 and 47 years with severe diarrhea and malabsorption, small bowel villous atrophy, no response to gluten-free diet, and circulating antienterocyte antibodies (6, 7). Only occasional case reports of adults with this disorder have appeared since, and similarly to pediatric cases, adultonset AIE has been associated with autoimmunity and immunoglobulin deficiency. AIE likely is but one manifestation of a more diffuse autoimmune disorder of the gastrointestinal system with heterogeneous manifestations including gastritis, colitis, hepatitis, and pancreatitis, as well as a variety of autoantibodies, such as anti–parietal cell antibodies and anti–goblet cell antibodies (4, 8, 9). León et al. recently coined the term generalized autoimmune gut disorder (GAGD) for those whose autoimmune gastrointestinal damage extends beyond the small intestine,