Celiac disease (CD) is a syndrome characterized by damage of the small inte
stinal mucosa caused by the gliadin fraction of wheat gluten and similar al
cohol-soluble proteins (prolamines) of barley and rye in genetically suscep
tible subjects. The presence of gluten in these subjects leads to self-perp
etuating mucosal damage, whereas elimination of gluten results in full muco
sal recovery. The clinical manifestations of CD are protean in nature and v
ary markedly with the age of the patient, the duration and extent of diseas
e, and the presence of extraintestinal pathologic conditions. In addition t
o the classical gastrointestinal form, a variety of other clinical manifest
ations of the disease have been described, including atypical and asymptoma
tic forms. Therefore, diagnosis of CD is extremely challenging and relies o
n a sensitive and specific algorithm that allows the identification of diff
erent manifestations of the disease. Serologic tests developed in the last
decade provide a noninvasive tool to screen both individuals at risk for th
e disease and the general population, However, the current gold standard fo
r the diagnosis of CD remains histologic confirmation of the intestinal dam
age in serologically positive individuals. The keystone treatment of CD pat
ients is a lifelong elimination diet in which food products containing glut
en are avoided.