Scholarlink

<p>Refractory celiac disease (RCD) is a&#xa0;rare but severe complication in patients with celiac disease and should only be considered after careful assessment of adherence to a&#xa0;gluten-free diet and exclusion of other causes of villous atrophy. Endoscopy, histology, immunopathology and flow cytometry are essential for establishing the diagnosis and for distinguishing between RCD type&#xa0;I and type&#xa0;II. Once the diagnosis is confirmed, a&#xa0;comprehensive nutritional evaluation is crucial and forms the foundation of clinical management. The path towards a&#xa0;targeted therapeutic approach for RCD is still long and complex. Open-capsule budesonide is considered the first line treatment in RCD type I, for which its efficacy has been demonstrated; however, it is associated with high recurrence rates after discontinuation of treatment, often necessitating long-term treatment. Second-line treatment with immunosuppressants has so far shown limited efficacy and has not been evaluated in large prospective studies. In RCD type&#xa0;II, the situation is even more challenging, as no currently available treatment can prevent the development of enteropathy-associated T‑cell lymphoma (EATL) by effectively eliminating aberrant intraepithelial lymphocytes. Nonetheless, purine analogues and stem cell transplantation can provide support in severe cases, while new drugs, particularly Janus kinase (JAK) inhibitors, have shown promising preliminary results.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Medikamentöse Therapieoptionen bei refraktärer Zöliakie

  • 期刊论文

  • 出版模式：

    开放获取

  • 发表日期：

    2026年3月9日

查看全文
  • Matilde Topa,
  • Maximilian Herter,
  • Federica Branchi

摘要

Refractory celiac disease (RCD) is a rare but severe complication in patients with celiac disease and should only be considered after careful assessment of adherence to a gluten-free diet and exclusion of other causes of villous atrophy. Endoscopy, histology, immunopathology and flow cytometry are essential for establishing the diagnosis and for distinguishing between RCD type I and type II. Once the diagnosis is confirmed, a comprehensive nutritional evaluation is crucial and forms the foundation of clinical management. The path towards a targeted therapeutic approach for RCD is still long and complex. Open-capsule budesonide is considered the first line treatment in RCD type I, for which its efficacy has been demonstrated; however, it is associated with high recurrence rates after discontinuation of treatment, often necessitating long-term treatment. Second-line treatment with immunosuppressants has so far shown limited efficacy and has not been evaluated in large prospective studies. In RCD type II, the situation is even more challenging, as no currently available treatment can prevent the development of enteropathy-associated T‑cell lymphoma (EATL) by effectively eliminating aberrant intraepithelial lymphocytes. Nonetheless, purine analogues and stem cell transplantation can provide support in severe cases, while new drugs, particularly Janus kinase (JAK) inhibitors, have shown promising preliminary results.