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Advances in the understanding and management of autoimmune enteropathy

机译：自身免疫性肠病的认识和治疗进展

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There have been real advances in understanding the pathogenesis of autoimmune enteropathy, including determination of specific autoantigens. The most important clinical association is with IPEX (X-linked immune polyendocrinopathy) syndrome, which is due to mutation in the Foxp.3 transcription factor, a molecule critical in generation of regulatory T cells. Association of non-IPEX autoimmune enteropathy with Tcell activation defects further point to impairment of Tcell tolerance mechanisms as the primary underlying cause of autoimmune enteropathy. This also explains the frequency of other autoimmune manifestations. The centrality of T cell responses in autoimmune enteropathy, rather than B cell autoantibody production, as previously thought, is further suggested by the finding of late-onset gut autoimmunity in APS-1 (autoimmune polyglandular syndrome-1), a condition where negative selection of Tcells within the thymus is disrupted due to mutation in the Aire (autoimmune regulator) gene. However, this form of autoimmune enteropathy is milder because the immune target is within entero-endocrine cells rather than absorptive enterocytes. There have also been important changes in management, with introduction of more potent immunoregulatory therapy, and more recently the use of bone marrow transplantation, which may theoretically offer hope of a cure in what frequently used to be a fatal condition.
机译：在了解自身免疫性肠病的发病机理方面已有真正的进展，包括确定特定的自身抗原。最重要的临床关联是IPEX（X连锁免疫多内分泌病）综合征，这是由于Foxp.3转录因子的突变所致，Foxp.3转录因子是调节性T细胞生成中的关键分子。非IPEX自身免疫性肠病与T细胞活化缺陷的相关性进一步指出，T细胞耐受性机制的损害是自身免疫性肠病的主要原因。这也解释了其他自身免疫表现的频率。 TPS反应在自身免疫性肠病中的中心作用，而不是先前认为的B细胞自身抗体的产生，是通过在APS-1（自身免疫性多腺综合征1）中发现迟发性肠道自身免疫性进一步提示的，这种情况是阴性选择由于Aire（自身免疫调节剂）基因突变，导致胸腺中T细胞的数量被破坏。但是，这种形式的自身免疫性肠病较轻，因为免疫靶标位于肠内分泌细胞内，而不是吸收性肠细胞内。随着更有效的免疫调节疗法的引入，管理方法也发生了重要变化，最近更是使用了骨髓移植，从理论上讲，这可能为治愈通常是致命的疾病提供希望。

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