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叙述性综述:乳糜泻:了解一种复杂的自身免疫性疾病。

Narrative review: celiac disease: understanding a complex autoimmune disorder.

作者信息

Alaedini Armin, Green Peter H R

机构信息

Department of Neurology and Neuroscience, Cornell University, New York, New York 10021, USA.

出版信息

Ann Intern Med. 2005 Feb 15;142(4):289-98. doi: 10.7326/0003-4819-142-4-200502150-00011.

DOI:10.7326/0003-4819-142-4-200502150-00011
PMID:15710962
Abstract

Celiac disease is a common autoimmune disorder that has genetic, environmental, and immunologic components. It is characterized by an immune response to ingested wheat gluten and related proteins of rye and barley that leads to inflammation, villous atrophy, and crypt hyperplasia in the intestine. The disease is closely associated with genes that code for human leukocyte antigens DQ2 and DQ8. Transglutaminase 2 appears to be an important component of the disease, both as a deamidating enzyme that can enhance the immunostimulatory effect of gluten and as a target autoantigen in the immune response. Sensitive and specific serologic tests, including those for anti-transglutaminase antibody, are facilitating fast and noninvasive screening for celiac disease. Thus, they are contributing to a more accurate estimate of the prevalence of the disease and its association with other disorders. Celiac disease is associated with increased rates of anemia, osteoporosis, cancer, neurologic deficits, and additional autoimmune disorders. A gluten-free diet is the mainstay of safe and effective treatment of celiac disease, although its effect on some of the extraintestinal manifestations of the disease remains to be determined.

摘要

乳糜泻是一种常见的自身免疫性疾病,具有遗传、环境和免疫等多种因素。其特征是对摄入的小麦麸质以及黑麦和大麦的相关蛋白质产生免疫反应,进而导致肠道炎症、绒毛萎缩和隐窝增生。该疾病与编码人类白细胞抗原DQ2和DQ8的基因密切相关。转谷氨酰胺酶2似乎是该疾病的一个重要组成部分,它既是一种能够增强麸质免疫刺激作用的脱酰胺酶,也是免疫反应中的一个自身抗原靶点。包括抗转谷氨酰胺酶抗体检测在内的敏感且特异的血清学检测,正在推动对乳糜泻进行快速且无创的筛查。因此,它们有助于更准确地估计该疾病的患病率及其与其他疾病的关联。乳糜泻与贫血、骨质疏松症、癌症、神经功能缺损以及其他自身免疫性疾病的发病率增加有关。无麸质饮食是乳糜泻安全有效治疗的主要方法,尽管其对该疾病某些肠外表现的影响仍有待确定。

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