Al-Toma Abdulbaqi, Verbeek Wieke H M, Mulder Chris J J
Department of Gastroenterology, VU University Medical Center, Amsterdam, The Netherlands.
J Gastrointestin Liver Dis. 2007 Mar;16(1):57-63.
True Refractory Coeliac Disease (RCD) is being currently defined as persisting or recurring villous atrophy with crypt hyperplasia and increased intraepithelial lymphocytes in spite of a strict gluten free diet for more than 12 months or when severe persisting symptoms necessitate intervention independent of the duration of the dietary therapy. Currently two categories of RCD are being recognized: type I without aberrant T-cells and type II with aberrant T-cells detected by immunophenotyping by flowcytometric analysis or immunohistology of the intestinal mucosa. Establishing the diagnosis of RCD requires exclusion of other causes of villous atrophy and malignancies that may complicate coeliac disease. In contrast to patients with a high percentage of aberrant T cells, patients with RCD type I seem to profit from an immunosuppressive treatment. In cases of RCD II with persistent clinical symptoms and/or high percentage of aberrant T cells in intestinal biopsies in spite of a corticosteroid treatment, more aggressive therapeutic schemes should be considered. However, no therapy seems to be curative in RCD II. Cladribine (2-CDA) seems to have some role in the management of these patients. More recently, high dose chemotherapy followed by autologous stem cell transplantation has been used in patients resulting in a dramatic improvement in the clinical, laboratory, histopathological and immunological parameters. This review provides an overview of the currently available diagnostic and therapeutic methods in a complicated form of coeliac disease.
真正的难治性乳糜泻（RCD）目前被定义为，尽管严格遵循无麸质饮食超过12个月，仍存在持续性或复发性绒毛萎缩、隐窝增生以及上皮内淋巴细胞增多的情况；或者当严重的持续性症状需要干预时，无论饮食治疗的持续时间长短。目前，RCD被分为两类：I型无异常T细胞，II型可通过肠道黏膜的流式细胞术免疫表型分析或免疫组织学检测到异常T细胞。确诊RCD需要排除其他可能导致绒毛萎缩的原因以及可能使乳糜泻复杂化的恶性肿瘤。与异常T细胞比例较高的患者不同，I型RCD患者似乎能从免疫抑制治疗中获益。对于II型RCD患者，尽管接受了皮质类固醇治疗，但如果肠道活检中仍有持续的临床症状和/或高比例的异常T细胞，则应考虑更积极的治疗方案。然而，似乎没有任何疗法能治愈II型RCD。克拉屈滨（2-CDA）似乎在这些患者的治疗中发挥了一定作用。最近，高剂量化疗后进行自体干细胞移植已应用于患者，使临床、实验室、组织病理学和免疫学参数有了显著改善。本综述概述了目前在这种复杂形式的乳糜泻中可用的诊断和治疗方法。
