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Defective innate immunity in inflammatory bowel disease: a Crohn's disease exclusivity?

机译:炎性肠病中先天性免疫缺陷:克罗恩病的排他性?

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PURPOSE OF REVIEW: This review summarizes the recent developments in support of the immunodeficiency model of Crohn's disease. RECENT FINDINGS: The demonstration of impaired acute inflammation in Crohn's disease provides a novel mechanism for its pathogenesis, with diminished macrophage cytokine production and neutrophil recruitment leading to reduced bacterial clearance. The innate immune response may be further overwhelmed by other factors. The mucosal barrier in Crohn's patients is disrupted, with abnormal ultrastructure as well as antibacterial defensin deficiency. Specific bacterial agents may contribute and one promising candidate, adherent-invasive Escherichia coli, has recently been described. An interaction between Nod2 and the autophagy system has been elucidated, with direct consequences for bacterial clearance, and the most recent genome-wide association study meta-analysis has extended the number of Crohn's disease susceptibility loci to 71. The spectrum of congenital immunodeficiency disorders recognized to develop Crohn's-like inflammatory bowel disease is also expanding. Conversely, no specific immunodeficiency has so far been observed in ulcerative colitis, in which the defect appears to be failure of inflammation termination and resolution. SUMMARY: Recent advances continue to highlight defects in innate immunity in Crohn's patients. Similar abnormalities may extend to other granulomatous disorders, but not diseases such as ulcerative colitis.
机译:审查的目的:这篇综述总结了支持克罗恩病免疫缺陷模型的最新进展。最近的发现:克罗恩病急性炎症受损的证明为其发病机理提供了一种新的机制,巨噬细胞细胞因子的产生减少,中性粒细胞募集减少,细菌清除率降低。先天性免疫反应可能会被其他因素进一步压倒。克罗恩病患者的粘膜屏障被破坏,超微结构异常以及抗菌素防御素缺乏。特定的细菌可能起作用,最近已经描述了一种有前途的候选物,即粘附侵袭性大肠杆菌。阐明了Nod2和自噬系统之间的相互作用,这直接影响了细菌清除,最近的全基因组关联研究荟萃分析已将克罗恩病易感基因座的数量扩展至71个。克罗恩氏样炎症性肠病的发展也在扩大。相反,迄今为止,尚未在溃疡性结肠炎中观察到特异性免疫缺陷,其中该缺陷似乎是炎症终止和消退失败。摘要:最近的进展继续突出了克罗恩病患者先天免疫的缺陷。类似的异常可能会扩展到其他肉芽肿性疾病,但不会扩展到溃疡性结肠炎等疾病。

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