Resumen de Refractory Celiac Disease

Luis Vaquero, Laura Arias, Santiago Vivas Alegre

• The main cause of failure to respond to a gluten-free diet (GFD) is persistent gluten ingeston,generally unnotced. The refractory celiac disease (RCD) diagnosis is established afer excludingother diseases, given the persistence of malabsorpton and villous atrophy. This situaton mayappear initally afer the disease diagnosis (primary) or afer the inital response, when symptomsrelapse despite strict adherence to a GFD (secondary). RCD comprises a heterogeneous group of patents, usually in adults, which share a fortunatelyuncommon cause of non-responsiveness to the GFD (<5% of the celiac populaton). Thedetecton changes in the intraepithelial lymphocyte populaton of the duodenal mucosa is offundamental importance. When these lymphocytes appear in a populaton that does not expressthe surface T-cell receptor (CD3 and CD8), this is a potentally aggressive form of CD with a higherpercentage of progression towards lymphoma (type II RCD). Therapy is based on an adequate nutritonal support and the use of cortcosteroids orimmunosuppressants (azathioprine and infiximab). The high risk of progression towards T celllymphoma in type II RCD demands the use of diferent therapeutc regimens. Although currentlyno treatment has clearly shown to be efectve in the long term, cladribine, immunotherapy withant-CD52 (or similar treatments) and autologous stem cell transplantaton are optons toconsider in the management of type II RCD. Antbodies that block interleukin-15 epithelialsecreton, which is a key molecule in the pathogenesis, may have potental as new therapies.