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Pronunciations celiac disease corticosteroid dermatitis dermatitis herpetiformis edema enteropathy gluten enteropathy herpetiformis lymphangiectasia lymphoma nontropical sprue prednisone steatorrhea

Celiac sprue (nontropical sprue, gluten enteropathy, celiac disease) is a hereditary intolerance to gluten, a protein found in wheat, barley, and oats, which causes characteristic changes in the lining of the small intestine, resulting in malabsorption.

• The intestinal lining becomes inflamed after a person ingests the protein gluten.
• Symptoms in adults include diarrhea, malnutrition, and weight loss; symptoms in children include abdominal bloating and bulky, very foul-smelling stools.
• The diagnosis is based on typical symptoms and examination of a tissue specimen removed from the lining of the small intestine.
• Most people do well if they maintain a gluten-free diet.

Celiac sprue affects as many as 1 out of 150 people in southwestern Ireland, 1 out of 300 people in Europe, and perhaps 1 out of 250 people in the United States, yet it is extremely rare in Africa, Japan, and China. There is a genetic component. About 10% of people with celiac sprue have a close relative with the disease. In this disease, gluten, a protein found in wheat and, to a lesser extent, barley, rye, and oats, stimulates the immune system to produce certain antibodies. These antibodies damage the inner lining of the small intestine, resulting in flattening of the villi. The resulting smooth surface leads to malabsorption of nutrients. However, the small intestine's normal brushlike surface and function are restored when the person stops eating foods containing gluten.

Symptoms

Some people develop symptoms as children. Others do not develop symptoms until adulthood. The severity of symptoms depends on how much of the small intestine is affected.

Adults with the more classic or typical form of the disease experience diarrhea, malnutrition, and weight loss. However, some people have no digestive symptoms at all. About 10% of people with celiac sprue develop a painful, itchy skin rash with small blisters—a disease called dermatitis herpetiformis (see Blistering Diseases: Dermatitis Herpetiformis).

In children, symptoms do not appear until foods containing gluten are introduced. Some children experience only mild upset stomach, whereas others develop painful abdominal bloating and have light-colored, unusually foul-smelling, bulky stools (steatorrhea).

The nutritional deficiencies resulting from malabsorption in celiac sprue can cause additional symptoms, which tend to be more prominent in children. Some children develop growth abnormalities, such as short stature. Anemia, causing fatigue and weakness, develops as a result of iron deficiency. Low protein levels in the blood can lead to fluid retention and tissue swelling (edema). Malabsorption of vitamin B₁₂ can lead to nerve damage, causing a pins-and-needles sensation in the arms and legs. Poor calcium absorption results in abnormal bone growth, a higher risk of broken bones, and painful bones and joints. Lack of calcium can also cause tooth discoloration and greater susceptibility to painful tooth decay. Girls with celiac sprue may not have menstrual periods because of a low production of hormones, such as estrogen.

Diagnosis

Doctors suspect the diagnosis when a person has the previously mentioned symptoms. Measurement of the level of specific antibodies produced when a person with celiac sprue consumes gluten is a helpful test. The diagnosis is confirmed by an initial microscopic examination of a biopsy specimen revealing flattened villi of the small intestine and by a subsequent improvement in the lining after the person stops eating foods containing gluten.

Prognosis and Treatment

Although most people do well if they avoid gluten, long-standing celiac sprue can be fatal in a small percentage of people who develop intestinal lymphoma. Whether strictly adhering to a gluten-free diet decreases the risk of long-term complications such as intestinal cancers or lymphoma is not known.

People with celiac sprue must exclude all gluten from their diet, because eating even small amounts may cause symptoms. The response to a gluten-free diet is usually rapid. Once gluten is avoided, the brushlike surface of the small intestine and its absorptive function return to normal. Gluten is so widely used in food products that people with celiac sprue need detailed lists of foods to be avoided and expert advice from a dietitian. Gluten is found, for example, in commercial soups, sauces, ice cream, and hot dogs.

Some people continue to have symptoms even when gluten is avoided. In such cases, either the diagnosis is incorrect or the disease has progressed to a condition called refractory celiac sprue. In refractory celiac sprue, treatment with corticosteroids, such as prednisoneSome Trade Names
DELTASONE
METICORTEN
, may help. In rare cases, if there is no response to either gluten withdrawal or drug treatment, intravenous feeding is needed. Sometimes children are seriously ill when first diagnosed and need a period of intravenous feeding before starting a gluten-free diet.

Last full review/revision December 2007 by Atenodoro R. Ruiz, Jr., MD