Detlef Schuppan1, Klaus-Peter Zimmer. 1. Institute of Translational Immunology, Johannes Gutenberg University, Mainz, and Division of Gastroenterology, Beth Israel deaconess Medical Center, Harvard Medical School, Boston, MA, USA, Center for Pediatric and Adolescent Medicine, Justus Liebig University, Gießen.
Abstract
BACKGROUND: Celiac disease is an inflammatory disease of, the small intestine with a prevalence of roughly 0.5%-1%. Its symptoms arise in response to gluten consumption by genetically predisposed persons (HLA-DQ2/8). The autoantigen tissue transglutaminase (TG2) plays an important role in the pathogenesis of celiac disease. METHOD: Selective review of pertinent literature, including guidelines from Germany and abroad. RESULTS: Celiac disease can present at any age with gastrointestinal or extraintestinal manifestations (e.g., malabsorption or Duhring's dermatitis herpetiformis); it can also be found in association with other (auto-)immune diseases, such as type 1 diabetes. Most cases are oligosymptomatic. The wide differential diagnosis includes food intolerances, intestinal infections, and irritable bowel syndrome, among other conditions. The definitive diagnosis requires the demonstration of celiac disease-specific autoantibody to TG2 (endomysium), which is over 90% sensitive and far over 90% specific, and the characteristic histologic lesions of the small-bowel mucosa and remission on a gluten-free diet. CONCLUSION: An understanding of celiac disease ought to inform everyday clinical practice in all medical disciplines, because this is a common condition with diverse manifestations that can be effectively diagnosed and easily treated for the prevention of both acute and long-term complications. Patients should follow a strictly gluten-free diet for life.
BACKGROUND: Celiac disease is an inflammatory disease of, the small intestine with a prevalence of roughly 0.5%-1%. Its symptoms arise in response to gluten consumption by genetically predisposed persons (HLA-DQ2/8). The autoantigen tissue transglutaminase (TG2) plays an important role in the pathogenesis of celiac disease. METHOD: Selective review of pertinent literature, including guidelines from Germany and abroad. RESULTS: Celiac disease can present at any age with gastrointestinal or extraintestinal manifestations (e.g., malabsorption or Duhring's dermatitis herpetiformis); it can also be found in association with other (auto-)immune diseases, such as type 1 diabetes. Most cases are oligosymptomatic. The wide differential diagnosis includes food intolerances, intestinal infections, and irritable bowel syndrome, among other conditions. The definitive diagnosis requires the demonstration of celiac disease-specific autoantibody to TG2 (endomysium), which is over 90% sensitive and far over 90% specific, and the characteristic histologic lesions of the small-bowel mucosa and remission on a gluten-free diet. CONCLUSION: An understanding of celiac disease ought to inform everyday clinical practice in all medical disciplines, because this is a common condition with diverse manifestations that can be effectively diagnosed and easily treated for the prevention of both acute and long-term complications. Patients should follow a strictly gluten-free diet for life.
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