Gastroenterology Department, Hospital de Vila Franca de Xira, 2600-009 Lisbon, Portugal.
Gastroenterology Department, Faculdade de Medicina, Lisbon University, 1649-028 Lisboa, Portugal.
Int J Mol Sci. 2024 Sep 26;25(19):10383. doi: 10.3390/ijms251910383.
Fewer than 1% of patients with celiac disease (CD) will develop refractory CD (RCD). As such, most gastroenterologists might never need to manage patients with RCD. However, all gastroenterologists must be familiarized with the basic concepts of RCD and non-responsive CD (NRCD), since it can present as a severe disease with high mortality, not only due to intestinal failure, but also due to progression to enteropathy-associated T cell lymphoma (EATL) and a higher susceptibility to life-threatening infections. The diagnostic workup and differential diagnosis with other causes of gastrointestinal symptoms and villous atrophy, as well as the differentiation between type I and II RCD, are complex, and may require specialized laboratories and reference hospitals. Immunosuppression is efficient in the milder RCDI; however, the treatment of RCDII falls short, with current options probably only providing transient clinical improvement and delaying EATL development. This review summarizes the current diagnostic and therapeutic approach for patients with RCD that all doctors that manage patients with CD should know.
不到 1%的乳糜泻（CD）患者会发展为难治性 CD（RCD）。因此，大多数胃肠病学家可能无需管理 RCD 患者。然而，由于 RCD 和非应答性 CD（NRCD）可能表现为一种死亡率高的严重疾病，不仅由于肠衰竭，而且由于进展为肠病相关 T 细胞淋巴瘤（EATL）和更易发生危及生命的感染，因此所有胃肠病学家都必须熟悉其基本概念。诊断工作以及与其他胃肠道症状和绒毛萎缩的鉴别诊断，以及 RCD 一型和二型的鉴别，都很复杂，可能需要专门的实验室和参考医院。免疫抑制在较轻的 RCDI 中有效；然而，RCDII 的治疗效果不佳，目前的选择可能只能提供短暂的临床改善并延迟 EATL 的发展。这篇综述总结了 RCD 患者的当前诊断和治疗方法，所有管理 CD 患者的医生都应该了解。
