Refractory Celiac Disease

Luis Vaquero Ayala, Laura Arias, Santiago Vivas


The main cause of failure to respond to a gluten-free diet (GFD) is persistent gluten ingestion, generally unnoticed. The refractory celiac disease (RCD) diagnosis is established after excluding other diseases, given the persistence of malabsorption and villous atrophy. This situation may appear initially after the disease diagnosis (primary) or after the initial response, when symptoms relapse despite strict adherence to a GFD (secondary). RCD comprises a heterogeneous group of patients, usually in adults, which share a fortunately uncommon cause of non-responsiveness to the GFD (<5% of the celiac population). The detection changes in the intraepithelial lymphocyte population of the duodenal mucosa is of fundamental importance. When these lymphocytes appear in a population that does not express the surface T-cell receptor (CD3 and CD8), this is a potentially aggressive form of CD with a higher percentage of progression towards lymphoma (type II RCD). Therapy is based on an adequate nutritional support and the use of corticosteroids or immunosuppressants (azathioprine and infliximab). The high risk of progression towards T cell lymphoma in type II RCD demands the use of different therapeutic regimens. Although currently no treatment has clearly shown to be effective in the long term, cladribine, immunotherapy with anti-CD52 (or similar treatments) and autologous stem cell transplantation are options to consider in the management of type II RCD. Antibodies that block interleukin-15 epithelial secretion, which is a key molecule in the pathogenesis, may have potential as new therapies.

Palabras clave

celiac, refractory

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