What is celiac sprue?

Causes and Symptoms

The most common symptoms of celiac sprue are diarrhea and weight loss. Patients develop an inflammatory reaction to gluten, a protein found in wheat, rye, oats, and barley. This inflammation causes flattening of the fingerlike projections in the small intestine known as "villi," thereby decreasing the surface area available for nutrient and fluid absorption. Thus, patients often have anemia (from malabsorption
of iron and folic acid) and bone disease (from malabsorption of calcium and vitamin D). Other symptoms of celiac sprue can include muscle weakness, infertility, epilepsy, and psychiatric illnesses, although the mechanisms underlying these manifestations are less clear. Some patients develop a blistering, itching
rash on the skin known as "dermatitis herpetiformis." Most patients come to medical attention at two years of age, after wheat is introduced into the diet. A small number of people develop the disease as adults. Research suggests that stress can trigger celiac sprue reactions, including extreme emotional stress, pregnancy, surgery, and infections.

Treatment and Therapy

The mainstay of therapy is elimination of gluten from the diet, which calms the inflammation and eventually allows the flattened villi to grow back. Symptoms usually improve within two weeks. Lack of improvement is most often the result of incomplete elimination of gluten. Alternative diagnoses—including infection, food allergies, inflammatory bowel disease, and lymphoma of the intestines—must also be considered.

The avoidance of gluten sounds straightforward, but it often requires significant dietary changes that must be rigorously followed throughout life. Most breads, pastas, and pastries must be avoided. Patients often benefit from counseling with a dietitian, since many processed foods contain gluten. Patients may also need to be screened for vitamin and mineral deficiencies. Folic acid supplementation is especially important for women of childbearing age. An x-ray to determine bone mineral density is sometimes helpful in patients with suspected vitamin D deficiency.

Perspective and Prospects

Celiac sprue is a genetic disease. It is known to be caused by a mutation on chromosome 6, and attempts to identify the specific gene further are ongoing. Testing to determine whether one has celiac sprue includes blood tests for antibodies against gliadin, the offensive component of gluten. About 1 percent of the global population suffers from celiac sprue. People at higher risk for celiac sprue include those who have diabetes mellitus, an autoimmune disease, Down syndrome, or anemia and those with a family member who has celiac sprue.

Bibliography

A.D.A.M. Medical Encyclopedia. "Celiac Disease." MedlinePlus, January 20, 2010.

Green, Peter H. R., and Rory Jones. Celiac Disease: A Hidden Epidemic. Rev. ed. New York: William R. Morrow, 2010.

Holmes, Geoffrey, C. Catassi, and Alessio Fasano. Celiac Disease. Abingdon, England: Health Press, 2009.

Korn, Danna. Kids with Celiac Disease: A Family Guide to Raising Happy, Healthy, Gluten-Free Children. Bethesda, Md.: Woodbine House, 2001.

Mahadov, Srihari, and Peter H. R. Green. "Celiac Disease: A Challenge for All Physicians." Gastroenterology & Hepatology 7, no. 8 (August, 2011): 554–556.

Parker, James N., and Philip M. Parker, eds. The Official Patient’s Sourcebook on Celiac Disease. San Diego, Calif.: Icon Health, 2002.

Shepard, Jules E. Dowler. The First Year: Celiac Disease and Living Gluten-Free—An Essential Guide for the Newly Diagnosed. Cambridge, Mass.: Da Capo Lifelong, 2009.

Tessmer, Kimberly A. Gluten-Free for a Healthy Life: Nutritional Advice and Recipes for Those Suffering from Celiac Disease and Other Gluten-Related Disorders. Franklin Lakes, N.J.: New Page Books, 2003.

Wood, Debra, and Daus Mahnke." Celiac Disease." Health Library, January 28, 2013.