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Celiac Disease: What It Is And Where It Can Be Found

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Ahmet Aybar, MD, FAAP

By Ahmet Aybar, MD, FAAP

Celiac Disease (CD) is one of the autoimmune disease processes affecting susceptible individuals with gluten sensitivity. Ingestion of gluten-containing grains, especially the gliadin fraction of wheat gluten, triggers intestinal damage. This eventually causes symptoms and can range from a silent disease to full blown CD.

The typical intestinal damage is seen in the duodenum and upper jejunum, affecting mucous membranes. The extent of this mucosal damage is highly variable and may involve the entire small bowel in some rare cases. The other characteristic finding is that the intestinal involvement may be patchy.

The intestinal damage is characterized by loss of absorptive surface of the small intestine, causing a variety of nutritional deficits in affected individuals. However, these changes completely resolve upon elimination of gluten from the patient’s diet.

Once considered a rare disorder, CD primarily affected individuals of European origin. In the past, diagnosis was made based on symptoms of the disease and subsequent confirmation by duodenal biopsy. However, with emergence of newer, highly sensitive and specific diagnostic tools, there has been a surge of clinically atypical or even silent forms of CD, affecting people all over the world.

CD is not only common in developed countries, but is increasingly reported in developing countries in North Africa, the Middle East, and Asia. CD contributes substantially to childhood morbidity and mortality in these countries.

As more sensitive diagnostic serologic tests have become available, it is now possible to evaluate the prevalence of CD (number of affected persons, including silent cases, in a defined population at a certain point in time). The prevalence of CD throughout the world seems to be more homogeneous. Furthermore, these screening tests showed that CD is one of the most frequent genetically based diseases occurring in 1 of 130-300 in the European population. Another interesting observation is that despite similar genetic backgrounds and environmental factors, the clinical presentation of CD may vary greatly between neighboring countries.

CD is the result of interaction between genetic and environmental factors (gluten-containing grains). Therefore if one evaluates the global distribution of these two factors, areas at risk can be identified. Europe is a high-risk region, but there have been limited epidemiologic studies in regions where CD was once considered rare. However, recent studies have shown that areas that are known to be at risk are South America, North Africa and Asia.

As seen in Europe, the clinical presentation of CD varies greatly in neighboring countries, which may explain the difference in prevalence previously reported. This difference stems from the fact that CD with typical gastrointestinal findings (in the intestine) is 15 times less common than CD with atypical findings (out of the intestine), making the diagnosis more challenging.

The intriguing question at present is whether environmental variables can influence the prevalence of CD, raising the possibility of primary prevention of this disorder.

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