Celiac disease: epidemiology, pathogenesis, diagnosis, and nutritional management.
Celiac disease (CD) is an inflammatory small intestinal disorder that can lead to severe villous atrophy, malabsorption, and malignancy. It is triggered by the gluten proteins of wheat, barley, and rye. All patients express the antigen-presenting molecules human leukocyte antigen-DQ2 (HLA-DQ2) and/or HLA-DQ8, which bind gluten peptides and thus activate destructive intestinal T cells. Patients with untreated CD have circulating IgA autoantibodies to the enzyme tissue transglutaminase (tTG), a component of endomysium. Testing for serum IgA tTG has a high predictive value. Therapy of CD is a lifelong gluten-free diet. Counseling by an expert dietitian and association with a celiac support group are important in helping the patient embark on a healthy gluten-free diet. Current research focuses on non-dietary therapies and treatment of refractory (diet-unresponsive) CD.
Schuppan
Detlef
D
Division of Gastroenterology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts 02215, USA. dschuppa@bidmc.harvard.edu
Dennis
Melinda D
MD
Kelly
Ciaran P
CP
D001323:Autoantibodies / Q000276:immunology; D001324:Autoantigens / Q000276:immunology; D002446:Celiac Disease / Q000175:diagnosis / Q000178:diet therapy / Q000453:epidemiology / Q000209:etiology; D005983:Glutens / Q000009:adverse effects* / Q000276:immunology / Q000378:metabolism; D006683:HLA-DQ Antigens / Q000235:genetics / Q000276:immunology; D006801:Humans; D007413:Intestinal Mucosa / Q000276:immunology / Q000473:pathology; D011503:Transglutaminases / Q000276:immunology*
D001323:Autoantibodies; D001324:Autoantigens; D006683:HLA-DQ Antigens; C053186:HLA-DQ2 antigen; D005983:Glutens; D011503:Transglutaminases