Wahab Peter J, Meijer Jos W R, Dumitra Daniela, Goerres Marije S, Mulder Chris J J
Dept of Gastroenterology, Rijnstate Hospital, PO Box 9555, 6800 TA Arnhem, The Netherlands.
Rom J Gastroenterol. 2002 Jun;11(2):121-7.
A continuing flow of new scientific developments concerning coeliac disease in the last decade asks for the formulation of a new concept of pathophysiology and clinical approach of the coeliac condition. Immunogenetic studies have shown a correlation of the disease to the HLA region on the short arm of chromosome 6. Immunological research has led to the concept of a T-cell driven immunologic response of the small intestine, with the identification of highly sensitive and specific antibodies, and in addition the understanding of the histopathology of coeliac disease has changed dramatically, initiated by the proposition of a spectrum of gluten sensitive enteropathy by Marsh in 1992. Clinical studies report a significant change in patient characteristics and epidemiology. The incidence of the disease has shifted to a majority of adult coeliacs and the disease may present with less severe symptoms of malabsorption while screening studies suggest an overall prevalence of up to 1 in 200-300. In the present paper (an update on histopathology) we specifically describe the work of our group in Arnhem, concerning the identification and validation of the spectrum of intestinal histopathology in gluten sensitive enteropathy, i.e. lymphocytic enteritis (Marsh I lesion), lymphocytic enteritis with crypthyperplasia (Marsh II lesion), and villous atrophy, subdivided in partial villous atrophy (Marsh IIIA), subtotal villous atrophy (Marsh IIIB) and total villous atrophy (Marsh IIIC). Special attention is given to a subgroup of "refractory coeliacs", including the identification of (pre-) malignant aberrant T-cells in the intestinal mucosa of these patients. The new data on immunogenetics, epidemiology, histo-pathology and patient characteristics point to a significant change of views on coeliac disease.
在过去十年中，关于乳糜泻的新科学进展不断涌现，这就要求制定一种新的病理生理学概念和乳糜泻病症的临床治疗方法。免疫遗传学研究表明，该疾病与6号染色体短臂上的HLA区域相关。免疫学研究催生了小肠T细胞驱动免疫反应的概念，同时鉴定出了高度敏感和特异的抗体，此外，1992年马什提出的一系列麸质敏感性肠病，引发了乳糜泻组织病理学认识的巨大变化。临床研究报告了患者特征和流行病学方面的显著变化。该疾病的发病率已转向以成年乳糜泻患者为主，并且疾病可能表现出不太严重的吸收不良症状，而筛查研究表明总体患病率高达200至300人中就有1人患病。在本文（组织病理学的最新进展）中，我们特别描述了我们在阿纳姆团队的工作，内容涉及麸质敏感性肠病肠道组织病理学谱系的鉴定和验证，即淋巴细胞性肠炎（马什I型病变）、伴有隐窝增生的淋巴细胞性肠炎（马什II型病变）以及绒毛萎缩，后者又细分为部分绒毛萎缩（马什IIIA型）、次全绒毛萎缩（马什IIIB型）和全绒毛萎缩（马什IIIC型）。我们特别关注了“难治性乳糜泻”亚组，包括在这些患者的肠道黏膜中鉴定（癌前）异常T细胞。免疫遗传学、流行病学、组织病理学和患者特征方面的新数据表明，人们对乳糜泻的看法发生了重大变化。
