Umetsu Sarah E, Brown Ian, Langner Cord, Lauwers Gregory Y
Department of Pathology, University of California, San Francisco, 505 Parnassus Avenue, Room M551A, Box 0102, San Francisco, CA, 94143-0102, USA.
Envoi Pathology, Kelvin Grove, Qld, Australia.
Virchows Arch. 2018 Jan;472(1):55-66. doi: 10.1007/s00428-017-2243-7. Epub 2017 Oct 11.
Autoimmune enteropathy (AIE) is a rare condition characterized by intractable diarrhea and immune-mediated injury of the intestinal mucosa. As the clinical and histopathologic manifestations of this disease are highly variable, its diagnosis is challenging for both clinicians and pathologists. In fact, the term autoimmune enteropathies is likely more appropriate since the clinicopathologic manifestations are observed in association with a heterogeneous group of disorders. The pathophysiology of AIE has not been fully elucidated. It appears to result from dysregulation of intestinal immunity and particularly in children, often presents in association with immunodeficiency. The overarching histopathologic changes seen in AIE include mucosal inflammation and epithelial injury, although this can manifest in the form of different patterns. Recognition of the clinical settings and of the various histologic patterns can aid the pathologist in establishing the correct diagnosis.
自身免疫性肠病（AIE）是一种罕见疾病，其特征为顽固性腹泻和肠道黏膜的免疫介导损伤。由于该疾病的临床和组织病理学表现高度多变，其诊断对临床医生和病理学家而言都具有挑战性。事实上，“自身免疫性肠病”这一术语可能更为恰当，因为临床病理表现与一组异质性疾病相关。AIE的病理生理学尚未完全阐明。它似乎是由肠道免疫失调导致的，尤其是在儿童中，常与免疫缺陷相关。AIE中所见的总体组织病理学变化包括黏膜炎症和上皮损伤，尽管其可表现为不同形式。认识临床背景和各种组织学模式有助于病理学家做出正确诊断。
