%0 Journal Article %T Celiac disease %A Wolfgang Holtmeier %A Wolfgang F Caspary %J Orphanet Journal of Rare Diseases %D 2006 %I BioMed Central %R 10.1186/1750-1172-1-3 %X Celiac disease (CD) in children and celiac sprue in adults are probably the same disorder with the same pathogenesis. The synonyms are: Coeliac disease (British spelling) ¨C Celiac sprue ¨C Nontropical sprue-Gluten-sensitive enteropathy ¨C Idiopathic steatorrheaCeliac disease is a chronic intestinal disease mostly associated with malabsorption caused by intolerance to gluten. It is characterized by immune-mediated enteropathy (villous flattening), resulting in maldigestion and malabsorption. Clinical and histological improvement can be obtained after withdrawal of dietary gluten.Celiac disease is characterized by malabsorption and villous atrophy. However, diseases other than CD can cause marked villous flattening and increased intraepithelial lymphocytes (IEL) [1]. Differential diagnosis is of special importance for subjects in whom CD is suspected and who have negative serology. The following diseases, which can have similar features, must be ruled out [1-4]:£¿ Tropical sprue£¿ Collagenous colitis£¿ Whipple's disease£¿ Giardiasis£¿ Viral enteritis£¿ AIDS£¿ Crohn's disease of the small intestine£¿ Small intestinal lymphoma£¿ Carbohydrate intolerance, cow's milk intolerance£¿ Autoimmune enteropathy£¿ Graft-vs-host disease£¿ Radiation damagePrevalence of clinically overt celiac disease varies from 1/270 in Finland to 1/5,000 in North America. However, since celiac disease can be asymptomatic, most subjects are not diagnosed or they can present with atypical symptoms. In epidemiological studies aimed to assess CD prevalence, large cohorts in North America and Europe were screened for highly-sensitive endomysium or tissue transglutaminase antibodies. Besides, they underwent subsequent small intestinal biopsies when antibody testing was positive. The CD prevalence was found to be much higher than expected. Approximately 1/100 to 1/500 were found positive for antibodies and had villous atrophy of the small intestine [5-10]. Thus, up to 1% of a western population tests positive for celi %U http://www.ojrd.com/content/1/1/3