Celiac Disease, Refractory Celiac Disease, and Tropical Sprue

Celiac disease, often also termed celiac sprue, is an immune-mediated disorder triggered by ingestion of wheat, barley, rye, and triticale gluten proteins in genetically predisposed individuals. The offending glutens induce a characteristic but variable and nonspecific lesion of the small intestinal mucosa that improves following gluten withdrawal. Patients with refractory celiac disease (RCD) have persistent or recurrent symptomatic malabsorption together with biopsy-documented enteropathy that has not responded to 6 to 12 months of strict gluten withdrawal. Tropical sprue is an illness that occurs in individuals living in tropical countries; clinical features include anorexia, diarrhea, and intestinal malabsorption, leading to weight loss accompanied by a characteristic although nonspecific lesion of the mucosa of the small intestine. This review addresses the epidemiology, etiology/genetics, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, complications, and prognosis of these related diseases. Figures show the celiac disease iceberg, duodenal mucosal surface seen at endoscopy, duodenal mucosal biopsies from a patient with celiac disease, higher magnification micrograph of the duodenal mucosal surface from a patient with untreated celiac disease, duodenal biopsies from a patient with refractory celiac disease, and duodenal biopsy obtained from an Indian expatriate with diarrhea, weight loss, and folate deficiency. Tables list selected extraintestinal manifestations of celiac disease, prevalence of celiac disease in selected conditions, disorders with duodenal lesions that may mimic those of celiac diseases, initial treatment for celiac disease, organizations that provide helpful information for gluten-intolerant patients, considerations in a patient not responding to a gluten-free diet, and features that help distinguish tropical sprue from celiac disease. This review contains 6 highly rendered figures, 7 tables, and 96 references.