Ryan B M, Kelleher D
Department of Gastroenterology and Clinical Medicine, St. James's Hospital and Trinity College, Dublin, Ireland.
Gastroenterology. 2000 Jul;119(1):243-51. doi: 10.1053/gast.2000.8530.
Celiac disease is a gluten-sensitive enteropathy, characterized by villous atrophy, which is reversed by gluten withdrawal. A minority of patients with celiac-like enteropathy are resistant to gluten-free diet, so-called refractory sprue, or unclassified sprue. Refractory sprue is a diagnosis of exclusion; all other causes of a celiac-like enteropathy must be eliminated before a diagnosis of refractory sprue can be made. Recent evidence suggests that refractory sprue comprises a heterogenous group of patients with diverse underlying causes. A small proportion of these patients seem to have an adult form of autoimmune enteropathy, characterized by the presence of antienterocyte antibodies. However, a larger group of patients with refractory sprue now seem to have a cryptic intestinal T-cell lymphoma, characterized by the presence of phenotypically abnormal, monoclonal intraepithelial lymphocytes, despite benign cytology. Current therapeutic options include nutritional support and immunosuppressive therapy, but response is variable. The prognosis of refractory sprue may be poor; patients may die of severe malabsorption, or through synchronous or metachronous development of an enteropathy-associated T-cell lymphoma. Based on this recent evidence, patients with refractory sprue should be screened for antienterocyte antibodies and have T-cell receptor and monoclonal antibody studies performed; this could facilitate identification of cases of adult-onset autoimmune enteropathy and those of cryptic T-cell lymphoma. Moreover, early recognition of the malignant nature of the intestinal infiltrate in some cases of refractory sprue could permit the development of novel chemotherapeutic regimens for this condition.
乳糜泻是一种麸质敏感性肠病，其特征为绒毛萎缩，戒食麸质后可逆转。少数乳糜泻样肠病患者对无麸质饮食有抵抗，即所谓的难治性口炎性腹泻或未分类口炎性腹泻。难治性口炎性腹泻是一种排除性诊断；在做出难治性口炎性腹泻的诊断之前，必须排除所有其他导致乳糜泻样肠病的原因。最近的证据表明，难治性口炎性腹泻包括一群病因各异的异质性患者。这些患者中有一小部分似乎患有成人型自身免疫性肠病，其特征是存在抗肠上皮细胞抗体。然而，现在看来，更大一组难治性口炎性腹泻患者患有隐匿性肠道T细胞淋巴瘤，其特征是存在表型异常的单克隆上皮内淋巴细胞，尽管细胞学表现为良性。目前的治疗选择包括营养支持和免疫抑制治疗，但反应不一。难治性口炎性腹泻的预后可能很差；患者可能死于严重吸收不良，或因肠病相关T细胞淋巴瘤的同步或异时发生而死亡。基于最近的这一证据，难治性口炎性腹泻患者应筛查抗肠上皮细胞抗体，并进行T细胞受体和单克隆抗体研究；这有助于识别成人发病的自身免疫性肠病病例和隐匿性T细胞淋巴瘤病例。此外，在某些难治性口炎性腹泻病例中早期识别肠道浸润的恶性性质，可能有助于为此病开发新的化疗方案。
