Schuppan Detlef, Zimmer Klaus-Peter
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.
Dtsch Arztebl Int. 2013 Dec 6;110(49):835-46. doi: 10.3238/arztebl.2013.0835.
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.
Selective review of pertinent literature, including guidelines from Germany and abroad.
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.
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.
乳糜泻是一种小肠炎症性疾病,患病率约为0.5%-1%。其症状是由遗传易感人群(HLA-DQ2/8)摄入麸质引发的。自身抗原组织转谷氨酰胺酶(TG2)在乳糜泻的发病机制中起重要作用。
对相关文献进行选择性综述,包括德国及国外的指南。
乳糜泻可在任何年龄出现胃肠道或肠外表现(如吸收不良或疱疹样皮炎);也可与其他(自身)免疫性疾病相关,如1型糖尿病。大多数病例症状较少。广泛的鉴别诊断包括食物不耐受、肠道感染和肠易激综合征等。确诊需要检测到针对TG2(肌内膜)的乳糜泻特异性自身抗体,其敏感性超过90%,特异性远超过90%,以及小肠黏膜的特征性组织学病变,且无麸质饮食后症状缓解。
所有医学学科的日常临床实践都应了解乳糜泻,因为这是一种常见疾病,有多种表现形式,可有效诊断且易于治疗,以预防急性和长期并发症。患者应终身严格遵循无麸质饮食。