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成人重度或部分绒毛萎缩性乳糜泻:一项对比研究。

Adult celiac disease with severe or partial villous atrophy: a comparative study.

作者信息

Malamut G, Matysiak-Budnik T, Grosdider E, Jais J-P, Morales E, Damotte D, Caillat-Zucman S, Brousse N, Cerf-Bensussan N, Jian R, Cellier C

机构信息

Department of Gastroenterology and Hepatology, hôpital européen Georges-Pompidou, 75015 Paris, France.

出版信息

Gastroenterol Clin Biol. 2008 Mar;32(3):236-42. doi: 10.1016/j.gcb.2008.02.011. Epub 2008 Mar 24.

DOI:10.1016/j.gcb.2008.02.011
PMID:18359595
Abstract

BACKGROUND AND AIMS

While severe villous atrophy (SVA) is the most typical histological feature in adult celiac disease (ACD), partial villous atrophy (PVA) is now also frequently found. So far, the impact of the severity of villous atrophy on the clinical presentation of ACD has been scarcely investigated. We aimed to compare the clinical, biological and immune features and outcomes in ACD patients presenting with PVA at diagnosis versus patients with SVA.

PATIENTS AND METHODS

Medical files of 48 patients with ACD diagnosed between 1992 and 2003 were retrospectively studied. The diagnosis was based on the presence of intestinal villous atrophy, with increases in intraepithelial lymphocytes and circulating celiac specific antibodies. Villous atrophy was classified as severe (subtotal and total) or partial. Symptoms, biological signs of malabsorption, immune markers, bone mineral density at diagnosis and response to gluten-free diet were recorded.

RESULTS

At diagnosis, ten patients (four M/six F) had PVA and 38 patients (five M/33 F) had SVA, with a median age of 54 and 33 years, respectively (p<0.05). Positivity for specific antibodies, HLA typing and frequency of autoimmune disease at diagnosis were similar in both PVA and SVA patients, as was their response to gluten-free diet. Diarrhea, malabsorption syndrome and osteopenia were independent of the degree of villous atrophy.

CONCLUSION

PVA was observed in 21% of patients with ACD. Except for their older age at diagnosis, patients with PVA presented with similar clinical, biological and immune characteristics and outcomes as did patients with SVA.

摘要

背景与目的

虽然严重绒毛萎缩(SVA)是成人乳糜泻(ACD)最典型的组织学特征,但现在也经常发现部分绒毛萎缩(PVA)。到目前为止,绒毛萎缩严重程度对ACD临床表现的影响鲜有研究。我们旨在比较诊断时表现为PVA的ACD患者与SVA患者的临床、生物学和免疫特征及预后。

患者与方法

回顾性研究了1992年至2003年间诊断的48例ACD患者的病历。诊断基于肠道绒毛萎缩的存在,以及上皮内淋巴细胞增多和循环中的乳糜泻特异性抗体。绒毛萎缩分为严重(完全和全部)或部分。记录症状、吸收不良的生物学体征、免疫标志物、诊断时的骨密度以及对无麸质饮食的反应。

结果

诊断时,10例患者(4例男性/6例女性)有PVA,38例患者(5例男性/33例女性)有SVA,中位年龄分别为54岁和33岁(p<0.05)。PVA和SVA患者诊断时特异性抗体阳性、HLA分型及自身免疫性疾病发生率相似,对无麸质饮食的反应也相似。腹泻、吸收不良综合征和骨质减少与绒毛萎缩程度无关。

结论

21%的ACD患者观察到PVA。除诊断时年龄较大外,PVA患者与SVA患者的临床、生物学和免疫特征及预后相似。

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