Introduction:

Celiac disease, also called celiac sprue, is a digestive disease in which eating gluten -- a protein found in bread, pasta, and other foods -- damages the small intestine. In people with celiac disease, gluten triggers an immune reaction in the intestine. That reaction damages the tiny, finger-like projections called villi, which help your intestine absorb nutrients. The villi become flattened and don’t work as well, so nutrients leave your body as part of your stool instead of being absorbed. Eventually you can become malnourished and deficient in vitamins and nutrients you need to stay healthy.

Gluten is found in wheat, barley, rye, spelt, kamut, and possibly oat products. Some vitamins and medicines also contain gluten. Many manufactured food products contain gluten in various forms under misleading names, like “modified food starch.” People with celiac disease must learn about all sources of gluten and read food labels thoroughly.

The disease can develop at any age. Although there is no cure, you can control symptoms by eating a gluten-free diet.

Signs and Symptoms:

The symptoms of celiac disease can vary from person to person. For example, one person may have constipation, another may have diarrhea, and yet a third may have no intestinal problems at all.

A partial listing of gastrointestinal symptoms:

• Abdominal pain
• Bloating, gas, indigestion
• Constipation
• Diarrhea, chronic or occasional
• Nausea and vomiting
• Stools that float, are foul smelling, bloody, or “fatty”

A partial listing of non-intestinal symptoms:

• Anemia
• Joint pain
• Osteoporosis
• Depression
• Fatigue
• Growth delay in children
• Irritability and behavioral changes
• Malnutrition
• Mouth ulcers
• Muscle cramps
• Unexplained short stature
• Skin problems (dermatitis herpetiformis)
• Unexplained weight loss (although people can be overweight or of normal weight upon diagnosis )

Causes:

Researchers don’t know the exact cause of celiac disease. Once thought rare, recent research suggests that as many as 1 in every 133 Americans has celiac disease. However, in the United States, only a small portion of people living with the disease are diagnosed.

Celiac disease is sometimes inherited. People who have a family history of celiac disease are at greater risk for developing the condition. It is most common in Caucasians and those of European ancestry. Women have it more often than men.

Risk Factors:

If someone in your immediate family has celiac disease, you have a 5 - 15% chance of getting it as well. The disease can be triggered for the first time after surgery, viral infection, severe emotional stress, pregnancy, or childbirth.

Diagnosis:

You will probably be referred to a gastroenterologist for a diagnosis. Your doctor may use blood tests to see if you have certain antibodies (proteins that are part of your immune system and that tend to be higher than normal in people with celiac disease). They include:

• Anti-gliadin (AGA)
• Anti-tissue transglutaminase (tTGA)
• IgA anti-endomysium antibodies (AEA)

To confirm the diagnosis, your doctor may use an endoscope (a small, flexible tube with a camera) to look into your small intestine and take a sample of tissue (biopsy).

A complete blood count (CBC) may indicate anemia. If anemia is detected, it is important to determine the cause.

An elevated alkaline phosphatase level may indicate bone loss, which is common before diagnosis.

Low cholesterol and albumin levels may reflect malabsorption and malnutrition.

Your doctor may order a follow-up biopsy or blood work several months after the diagnosis and treatment. These serve as a final confirmation of the disease.

Although a gluten-free diet is the treatment for celiac disease, it is important not to eat a gluten-free diet before you go to see a doctor for diagnosis. Eating such a diet may cause your blood tests and biopsy to appear normal.

Preventive Care:

No one knows how to prevent celiac disease. However, knowing if you are at higher risk (such as having a family member with the disorder) may increase the chance of early diagnosis and treatment.

In the United States, people without symptoms are not routinely screened for celiac disease. Antibody screening tests may not be reliable in young children.

Treatment:

You will need to eat a gluten-free diet for the rest of your life. This allows the intestinal villi to heal.

Completely eliminate foods, beverages, and medications that contain wheat, barley, rye, and possibly oats. Grains that are gluten-free when grown include buckwheat, quinoa, and amaranth. However, they may be contaminated with gluten when they are processed, so check labels carefully to make sure they say “manufactured in a gluten-free facility.”

Even a small amount of gluten may cause damage to your intestine -- even if it does not cause any symptoms. That’s why sticking to the diet is so important.

Read food and medication labels carefully to look for "hidden" sources of gluten. Since wheat and barley grains are found abundantly in the American diet, the treatment is challenging but can be done with education and planning.

Do NOT start a gluten-free diet before being diagnosed with celiac disease. Doing so will alter future testing for the disease.

If you are not able to absorb enough of some vitamins and nutrients, your doctor may prescribe vitamin and mineral supplements. Occasionally, doctors may prescribe corticosteroids (such as prednisone) for short-term use or if you have refractory sprue.

Following a well-balanced, gluten-free diet is generally the only treatment required to relieve symptoms of celiac disease. Several studies have also found that sticking to such a diet helps reduce the risk of intestinal lymphoma.

After you are diagnosed, you may want to consult with a registered dietitian with a specialty in celiac disease and the gluten-free diet. Joining a local and national support group can also help you cope with the disease and diet. Although the diet is restrictive, you can still enjoy many foods that are gluten-free:

• Meat, poultry, fish (not with breading or marinated)
• Fruits
• Vegetables
• Rice
• Flours made from rice, soy, potato, or corn

See also: Nutrition.

Prognosis/Possible Complications:

Removing all gluten from your diet is the most important thing you can do to stay healthy. If you follow a gluten-free diet for the rest of your life, you can expect to lead a long, healthy life (as long as permanent damage did not occur before diagnosis).

When untreated, celiac disease can cause life-threatening complications. A delayed diagnosis or failure to follow the diet puts you at risk for developing other conditions.

Complications can include:

• Malnutrition
• Osteoporosis or osteomalacia (softening of the bone)
• Dermatitis herpetiformis (a burning, itching, blistering rash)
• Intestinal cancer (Intestinal lymphoma is up to 40 times more common in patients with celiac disease than in those without the disease.)
• Seizures
• Nerve damage (peripheral neuropathy)
• Lactose intolerance

Other Considerations:

Because they may not be able to absorb nutrients properly, untreated pregnant women with celiac disease may be at higher risk of having a miscarriage or a baby born with birth defects such as neural tube defects.

Alternative Names:

Sprue; Nontropical sprue; Gluten intolerance; Gluten-sensitive enteropathy