World Gastroenterology Organisation Global Guidelines on Celiac Disease


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CONTENTS1 Definitions2 Key points3 Epidemiology4 Diagnosis of celiac disease5 Management of celiac diseaseDEFINITIONSCeliac disease (CD) is a chronic, immunologically determined form of enteropathy affecting the small intestine in genetically predisposed children and adults. It is precipitated by the ingestion of gluten-containing foods.1,2 It is also referred to as celiac sprue, gluten-sensitive enteropathy, or nontropical sprue.Gluten can be defined as the rubbery protein mass that remains when wheat dough is washed to remove starch.3 The major protein fractions of gluten, gliadin and glutenin are storage proteins in wheat. They are present in wheat, rye, and barley and gives the dough its desired baking properties. Gluten is widely used as an ingredient in food processing.KEY POINTSGluten and gluten-related proteins present in wheat, rye, and barley are the causative external antigens of CD.The prevalence of CD in an adult population varies between roughly 1 in 100 and 1 in 300 in most parts of the world.First-degree and (to a lesser extent) second-degree relatives have an increased risk for CD. Its clinical presentation varies widely, and the onset of the disease or symptoms may occur at any time in life.Many patients with CD have few symptoms or present atypically, whereas a minority of patients have malabsorption (classical CD).Patients with active CD have an increased risk of complications, including death, in comparison with the general population. However, this excess rate of major complications seems to return to normal after 3 to 5 years on a strictly gluten-free diet.Key Diagnostic Findings Include:Histopathologic changes in an intestinal biopsy, characterized by crypt hyperplasia, intraepithelial lymphocytosis, and destruction of the surface epithelial liningEvidence that the small bowel enteropathy is dependent on gluten shown by positive CD-specific antibodies and/or clinical and/or histologic improvement in response to a gluten-free dietSerological Tests Can:Confirm CD in patients with a demonstrated characteristic enteropathyScreen for individuals at riskIdentify patients in whom biopsy may be warranted.Be used to identify gluten consumption during follow-up in diagnosed patientsThe presence of autoantibodies directed against transglutaminase-2 suggests that CD has an autoimmune component.4 In adults, CD is diagnosed on average >10 years after the first symptoms appear.5Patients with CD should not eat products containing wheat, rye, or barley. Patients usually need to follow a strictly gluten-free diet for the rest of their lives.6 Oats may be consumed, but they are very often contaminated by wheat, and pure oats are often not available. A small subgroup of patients with CD (<5%) may also be intolerant to pure oats.7EPIDEMIOLOGYIntroductionCD is common throughout the world and affects around 1 in 100 to 1 in 300 of the population.8,9 This prevalence is significantly higher than that recognized 20 years ago.10,11 The epidemiology of CD has iceberg characteristics that there are far more undiagnosed cases (below the waterline) than diagnosed cases (above the waterline).A key study by Fasano et al12 in 2003 found that the prevalence of CD was as follows:At risk, first-degree relatives: 1 in 10At risk, second-degree relatives: 1 in 39At risk, symptomatic patients: 1 in 56Groups not at risk: 1 in 100EthnicityEarly epidemiology studies regarded CD as a disease of individuals with white ancestry, mainly located in Europe and North America.13,14 However, although there is a lack of worldwide epidemiological information, further studies in other areas of the world have shown a similar prevalence.

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