title: [Refractory celiac disease].
abstract: The main cause of lack of response to a gluten-free diet is continued, usually inadvertent, gluten intake. Diagnosis of refractory celiac disease is established on the basis of exclusion of other disorders, persistence of malabsorption and villous atrophy. Refractory celiac disease affects a heterogeneous group of patients, usually adults and, fortunately, is infrequent (<5% of the population). Detection of alterations in the intraepithelial lymphocyte population is essential for diagnosis. Some alterations in these lymphocytes, such as the absence of T cell surface receptor expression (CD3 and CD), indicate an aggressive form of the disease with the potential for malignant transformation (type II refractory celiac disease). Treatment is based on adequate nutritional support and on the use of corticosteroids and/or immunosuppressive agents (mainly azathioprine and infliximab). Because of the high risk of progression to intestinal T cell lymphoma, patients diagnosed with type 2 refractory disease require different -generally more aggressive- therapeutic strategies. At present, no treatment has been demonstrated to be effective in the long term, but two options that should be considered in type II disease are immunotherapy with anti-CD52 or similar agents, and autologous bone marrow transplantation. Trials with antibodies that block epithelial secretion of interleukin-15, a key molecule in the pathogenesis of the disease, are promising.
authors.affiliation: Servicio de Aparato Digestivo, Hospital de León, León, España. svivasa@medynet.com
authors.forename: Santiago
authors.initials: S
authors.lastname: Vivas Alegre
authors.forename: José María
authors.initials: JM
authors.lastname: Ruiz de Morales
mesh_terms: D000465:Algorithms; D002446:Celiac Disease / Q000145:classification / Q000209:etiology / Q000628:therapy*; D005983:Glutens; D006801:Humans; D011379:Prognosis; D017211:Treatment Failure
chemical_list: D005983:Glutens