This is a common disease in which the lining of the small intestine is damaged in response to ingestion of gluten and similar proteins , which are found in wheat, rye, oats, barley, and other grains (including hybrid grains, such as triticale).

Alternative Names

Sprue; Nontropical sprue; Gluten intolerance

Causes, incidence, and risk factors

The exact cause of celiac disease is unknown. There is evidence that the disorder is inherited since it tends to run in families. The disorder may be caused by an abnormal immune response to proteins found in grains, particularly gluten and the related protein gliadin. The intestines contain projections (called villi) that normally absorb nutrients. Celiac disease causes these villi to become flattened and lose the ability to absorb nutrients. Weight loss , anemia , and vitamin deficiencies may occur as a result of this malabsorption (inadequate absorption of nutrients from the intestinal tract). Symptoms appear in babies within 6 months of introducing food containing gluten into the diet. The disease also affects children and adults. As many as 1 in 250 people may have celiac disease and some studies estimate that it is even more common, at 1 in 150. Most people with the disease do not know they have it, as only about 1 in 2,500 is diagnosed. Risk factors are a genetic or familial tendency toward the disease. The disorder is most common in Caucasians and those with Northern and Southern European ancestry. Approximately 70% of reported cases occur in women. Diseases that can be associated with celiac disease include lactose intolerance , dermatitis herpetiformis (a burning, itching , blistering rash), insulin-dependent diabetes mellitus (IDDM) , systemic lupus erythematosus , thyroid disease , and autoimmune disorders .

Signs and tests

Typically, most people with celiac disease will have symptoms of

malabsorption such as diarrhea . However, some will have bone disease , anemia , or other conditions without diarrhea. Compression fractures of the back , kyphoscoliosis (see scoliosis ), or other signs of bone disease may be present. Steatorrhea ("fatty" diarrhea, or stools that can be foul smelling and unusually colored) is common. Dental examination may show changes in the teeth. In fact, some cases of celiac disease are suspected by a dentist because of the changes in the enamel of the teeth, which include symmetrical (the same on both sides) changes in the tooth color and surface texture. A CBC often indicates anemia. Two relatively new blood tests have improved the ability to accurately diagnose this disorder: Both the IgA antiendomysial antibody and the antigliadin antibodies are sensitive and specific for the diagnosis of celiac disease. An EGD ( endoscopy ) and small bowel biopsy , particularly biopsy of the jejunum (the part of the small intestine most often affected), will show an abnormal intestinal lining. Serial biopsies may be performed before and after a gluten-free diet . Improvement of the findings of biopsy (or improvement of symptoms) after a gluten- and gliadin-free diet is considered highly indicative of celiac disease.

Treatment

A life-long gluten-free diet is required. This allows the intestinal villi to heal. Foods that contain wheat, rye, oats, and barley must be eliminated from the diet. Food and drug labels should be read carefully to look for "hidden" sources of gluten. This often includes the words "vegetable protein " or "plant protein" (such as "tvp" -- textured vegetable protein). Other hidden sources of gluten include grain derivatives such as malt, modified food starch, soy sauce (some types), grain vinegar (or "distilled vinegar"), and some binders, fillers, and flavorings. Vitamin and mineral supplements may be needed to correct nutritional deficiencies. Sometimes, corticosteroids (such as prednisone) may also be required. The stress of a chronic disorder such as celiac disease can sometimes be helped by joining a support group where members share common experiences and problems.

Expectations (prognosis)

Untreated, the disorder can cause life-threatening complications. Symptoms usually disappear within several weeks after the person begins a gluten-free diet . The gluten-free diet must be followed continuously or the symptoms will return.

Complications

Vitamin and mineral deficiencies such as rickets and osteomalacia are common complications. Other complications include pancreas disorders, damage to the nerves, infertility , and miscarriage . People with celiac disease may be at a greater risk for lymphoma and intestinal cancers.

Calling your health care provider

Call your health care provider if symptoms develop that are suggestive of celiac disease. An appointment with a nutritionist or registered dietician may be helpful in determining dietary guidelines and finding safe foods. Many communities have support groups such as the Celiac Disease Foundation; these groups are also a good source of information about proper diet for those with the condition.

Prevention

Because the exact cause is unknown, there is no known way to prevent the development of celiac disease. However, awareness of risk factors (such as a family member with the disorder) may increase the chance of early diagnosis and treatment.