Although continued ingestion of gluten is the most frequent cause of non-responsive celiac disease, other considerations include associated or complicating conditions (small bowel bacterial overgrowth, carbohydrate malabsorption, microscopic colitis) or an incorrect initial diagnosis. Failure to respond to a gluten-free diet or recurrence of symptoms on a gluten-free diet, particularly in an older patient, should also prompt a search for lymphoma provided that ingestion of gluten has been excluded as the cause of the symptoms. Refractory sprue is a presentation of celiac disease characterized as being unresponsive to dietary therapy, usually requiring immune modulating therapy and associated with a poor prognosis . A subset of these patients with a band of subepithelial collagen present in small intestinal histological specimens is referred to as having collagenous disease. Other variants include structuring or ulcerative forms. It is thought that chronic stimulation of T cells leads to clonal expansion of IEL characterized by T cell TCR-7 gene rearrangements . Chromosomal abnormalities have been demonstrated in refractory sprue and in enteropathy associated
T cell lymphoma (EATCL) including partial trisomy of the 1q region in refractory sprue and gain of chromosome 1q and loss of heterozygosity at chromosome 9p21 in EATCL . Uncontrolled studies show corticosteroids including budesonide, immunosuppressive agents and infliximab to be of benefit in refractory celiac disease [60, 61]. Elemental enteral or parenteral nutrition may be required to manage these patients. These patients should be evaluated for the development of EATCL as described below.
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