Refractory celiac disease

Celiac disease is a gluten-sensitive enteropathy, characterized by villous atrophy, which is reversed by gluten withdrawal. A minority of patients wit h celiac-like enteropathy are resistant to gluten-free diet, so-called refr actory sprue, or unclassified sprue, Refractory sprue is a diagnosis of exc lusion; all other causes of a celiac-like enteropathy must be eliminated be fore a diagnosis of refractory sprue can be made. Recent evidence suggests that refractory sprue comprises a heterogenous group of patients with diver se underlying causes. A small proportion of these patients seem to have an adult form of autoimmune enteropathy, characterized by the presence of anti enterocyte antibodies. However, a larger group of patients with refractory sprue now seem to have a cryptic intestinal T-cell lymphoma, characterized by the presence of phenotypically abnormal, monoclonal intraepithelial lymp hocytes, despite benign cytology. Current therapeutic options include nutri tional support and immunosuppressive therapy, but response is variable. The prognosis of refractory sprue may be poor; patients may die of severe mala bsorption, or through synchronous or metachronous development of an enterop athy-associated T-cell lymphoma. Based on this recent evidence, patients wi th refractory sprue should be screened for antienterocyte antibodies and ha ve T-cell receptor and monoclonal antibody studies performed; this could fa cilitate identification of cases of adult-onset autoimmune enteropathy and those of cryptic T-cell lymphoma. Moreover, early recognition of the malign ant nature of the intestinal infiltrate in some cases of refractory sprue c ould permit the development of novel chemotherapeutic regimens for this con dition.