Refractory Sprue
A small percentage of patients diagnosed with celiac disease fail to have a long-term favorable response to a strict gluten-free diet. These individuals continue to have symptoms and histologic abnormalities or have symptomatic relapse despite treatment with gluten-free diet and ultimately develop a potentially life-threatening disease known as refractory sprue. Based on data reported in recent studies, the majority of patients with refractory sprue have abnormal intraepithelial lymphocytes and a poor prognosis. The current hypothesis is that refractory sprue represents a transition state to intestinal lymphoma. The true prevalence of this disease entity is unknown. Future multicenter trials are therefore warranted to estimate its prevalence and establish clear therapeutic guidelines. I ntroduction Celiac disease (CD) is a gluten-sensitive enteropathy with a broad spectrum of clinical manifestations. Based on current knowledge, the initial step in disease pathogenesis is the deamidatio