Corazza G R, Biagi F, Volta U, Andreani M L, De Franceschi L, Gasbarrini G
Department of Internal Medicine, University of L'Aquila, Italy.
Lancet. 1997 Jul 12;350(9071):106-9. doi: 10.1016/S0140-6736(97)01042-8.
Autoimmune enteropathy is a condition described in children and characterised by villous atrophy, which is unresponsive to any dietary restrictions, and by the presence of enterocyte autoantibodies. We report two adult patients who fulfilled all the criteria for the diagnosis of this disorder.
Over the past 5 years we have seen four adult patients (all women, median age 51.5 [range 38-64] years) with subtotal villous atrophy, which was unresponsive to a gluten-free diet. The patients were HLA-DQ2 positive. IgA antigliadin and antiendomysial antibodies were not found in any of the patients. We did an indirect immunofluorescence search for enterocyte autoantibodies on monkey jejunum and for other autoantibodies for all four patients.
Of the four patients, two were positive for enterocyte autoantibodies and one of these two patients was positive for antiactin, antiparietal cell, and antithyroid microsomal autoantibodies.
To the best of our knowledge the two patients affected by severe enteropathy, who had never responded to any exclusion diet, and who were positive for enterocyte autoantibodies are the first cases of autoimmune enteropathy described in adults. We propose that adult patients whose disorders are unresponsive to a gluten-free diet should be tested for enterocyte autoantibodies.
自身免疫性肠病是一种在儿童中描述的疾病，其特征为绒毛萎缩，对任何饮食限制均无反应，且存在肠上皮细胞自身抗体。我们报告了两名符合该疾病诊断所有标准的成年患者。
在过去5年中，我们诊治了4名成年患者（均为女性，中位年龄51.5岁[范围38 - 64岁]），他们患有广泛性绒毛萎缩，对无麸质饮食无反应。这些患者HLA - DQ2呈阳性。所有患者均未检测到IgA抗麦醇溶蛋白和抗肌内膜抗体。我们对所有4名患者进行了间接免疫荧光检测，以检测猴空肠上的肠上皮细胞自身抗体以及其他自身抗体。
4名患者中，2名肠上皮细胞自身抗体呈阳性，其中1名患者抗肌动蛋白、抗壁细胞和抗甲状腺微粒体自身抗体也呈阳性。
据我们所知，这两名患有严重肠病且对任何排除饮食均无反应、肠上皮细胞自身抗体呈阳性的患者是首次报道的成年自身免疫性肠病病例。我们建议，对无麸质饮食无反应的成年患者应检测肠上皮细胞自身抗体。
