Refractory celiac disease.
Refractory celiac disease.
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DOI：
10.1007/s11894-007-0049-5
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发表时间：
2007-10-01
影响因子：
--
通讯作者：
Kelly, Ciaran P
中科院分区：
文献类型：
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作者：
Abdallah, Hani;Leffler, Daniel;Kelly, Ciaran P
乳糜泻（CD）是一种小肠炎性疾病，其特征在于在携带人类白细胞抗原（HLA）-DQ 2或-DQ 8的遗传易感个体中由摄入小麦面筋或相关黑麦和大麦蛋白引发的免疫介导的肠病。无反应CD（NRCD）是一种临床诊断，定义为尽管坚持无麸质饮食至少6个月，但CD的典型体征、症状和/或实验室异常持续存在。NRCD的一个原因是难治性CD（RCD），其定义为尽管严格停用谷蛋白至少6个月，但小肠活检中严重绒毛萎缩持续存在，且没有其他病理学证据。虽然罕见，但在已确诊CD的个体中应怀疑RCD，这些个体对严格的无麸质饮食没有主要或次要反应，特别是如果他们表现出显着的体重减轻。必须进行全面的评估，以区分RCD与其他原因的NRCD。RCD可分为I型或II型。与II型相比，I型RCD具有更有利的预后，并且通常可以通过营养补充和可能的低水平免疫抑制治疗来管理。II型RCD预后不良，更有可能发展为危及生命的营养不良或肠道T细胞淋巴瘤。免疫抑制剂和最近的自体干细胞移植已被用于治疗II型RCD。
Celiac disease (CD) is a small intestinal inflammatory disorder characterized by an immune-mediated enteropathy triggered by the ingestion of wheat gluten or related rye and barley proteins in genetically predisposed individuals carrying the human leukocyte antigens (HLA)-DQ2 or -DQ8. Nonresponsive CD (NRCD) is a clinical diagnosis defined by the persistence of signs, symptoms, and/or laboratory abnormalities typical of CD despite adherence to a gluten-free diet for at least 6 months. One cause for NRCD is refractory CD (RCD), defined as the persistence of severe villous atrophy on small intestinal biopsy despite strict gluten withdrawal for at least 6 months with no evidence of other pathology. Although rare, RCD should be suspected in individuals with an established diagnosis of CD who fail to respond primarily or secondarily to a strict gluten-free diet, particularly if they manifest significant weight loss. A thorough evaluation must be performed to distinguish RCD from other causes of NRCD. RCD may be categorized into type I or type II. Type I RCD has a more favorable prognosis compared with type II and can often be managed with nutritional supplementation and possibly low level immunosuppressive therapy. Type II RCD carries a poor prognosis and is more likely to progress to life-threatening malnutrition or intestinal T-cell lymphoma. Immunosuppressive agents and, more recently, autologous stem cell transplant have been used to treat type II RCD.