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原发性胆汁性肝硬化患者的麸质敏感性

Gluten sensitivity in patients with primary biliary cirrhosis.

作者信息

Niveloni S, Dezi R, Pedreira S, Podestá A, Cabanne A, Vazquez H, Sugai E, Smecuol E, Doldan I, Valero J, Kogan Z, Boerr L, Mauriño E, Terg R, Bai J C

机构信息

Clinical Department, Hospital de Gastroenterología Dr Carlos Bonorino Udaondo, Universidad del Salvador, Buenos Aires, Argentina.

出版信息

Am J Gastroenterol. 1998 Mar;93(3):404-8. doi: 10.1111/j.1572-0241.1998.00404.x.

Abstract

OBJECTIVE

Whereas celiac disease and primary biliary cirrhosis have been reported to coexist in the same patient, the frequency of this relationship has not been clarified. Nowadays, the concept of celiac disease has been extended from that of a severe enteropathy to a broader concept of gluten-driven intestinal immunological response. In this study we assessed features of gluten sensitivity in a cohort of patients with primary biliary cirrhosis.

METHODS

Ten patients with primary biliary cirrhosis were evaluated a mean of 2 yr after diagnosis. The following features of gluten sensitivity were assessed: serum antigliadin and endomysial antibodies, small bowel histology (degree of atrophy and quantitative histological parameters), the presence of the typical celiac HLA genotype (DQ2), and intraepithelial lymphocyte response in the rectal mucosa after local gluten instillation (rectal gluten challenge).

RESULTS

Overall, three patients presented evidence of gluten sensitivity. All three had abnormal titers of antigliadin antibody type IgA and one was positive for endomysial antibody. Two patients had partial villous atrophy. The rectal gluten challenge showed a celiac-like response, evidenced by an increase in intraepithelial lymphocyte infiltration after gluten exposure, in the three patients. The characteristic celiac HLA genotypes (DQA1 0501 and DQB1 0201) were identified in three patients. One of them also exhibited other features of gluten sensitivity. However, despite evidence of gluten intolerance, patients had minimal or no symptoms characteristic of celiac disease.

CONCLUSION

We detected features of gluten sensitivity in a high proportion of patients with primary biliary cirrhosis. Further studies should be performed to elucidate the clinical significance of this association.

摘要

目的

尽管有报道称乳糜泻与原发性胆汁性肝硬化可在同一患者中共存,但这种关系的发生率尚未明确。如今,乳糜泻的概念已从一种严重的肠病扩展为麸质驱动的肠道免疫反应这一更广泛的概念。在本研究中,我们评估了一组原发性胆汁性肝硬化患者的麸质敏感性特征。

方法

对10例原发性胆汁性肝硬化患者在诊断后平均2年进行评估。评估了以下麸质敏感性特征:血清抗麦醇溶蛋白和抗肌内膜抗体、小肠组织学(萎缩程度和定量组织学参数)、典型乳糜泻HLA基因型(DQ2)的存在情况,以及局部注入麸质后直肠黏膜中的上皮内淋巴细胞反应(直肠麸质激发试验)。

结果

总体而言,3例患者有麸质敏感性证据。所有3例患者的抗麦醇溶蛋白抗体IgA型滴度均异常,1例抗肌内膜抗体呈阳性。2例患者有部分绒毛萎缩。直肠麸质激发试验显示,这3例患者在接触麸质后上皮内淋巴细胞浸润增加,呈现出类似乳糜泻的反应。3例患者鉴定出特征性的乳糜泻HLA基因型(DQA1 0501和DQB1 0201)。其中1例还表现出其他麸质敏感性特征。然而,尽管有麸质不耐受的证据,但患者具有极少或没有乳糜泻的典型症状。

结论

我们在高比例的原发性胆汁性肝硬化患者中检测到麸质敏感性特征。应开展进一步研究以阐明这种关联的临床意义。

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