Introduction:

Celiac disease, also called celiac sprue, is an inherited disease that damages the small intestine and interferes with digestion. People with this disease cannot tolerate a protein called gluten. As a result, they have trouble absorbing nutrients from food. 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 educated themselves well regarding all possible sources of gluten.

The small intestine is lined with fingerlike projections called villi, which help absorb nutrients. In celiac disease, these villi become flattened, so they don't work as well and the person becomes malnourished.

The disease can develop at any age.

Signs and Symptoms:

The symptoms of celiac disease can vary significantly 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
• Abdominal distention, bloating, gas, indigestion
• Constipation
• Decreased appetite (may also be increased or unchanged)
• Diarrhea, chronic or occasional
• Lactose intolerance (common upon diagnosis; usually resolves following treatment)
• Nausea and vomiting
• Stools that float, are foul smelling, bloody, or “fatty”
• Unexplained weight loss (although people can be overweight or of normal weight upon diagnosis)

A partial listing of non-intestinal symptoms:

• Anemia
• Bone and joint pain
• Bone disease (osteoporosis, kyphoscholiosis, fracture)
• Breathlessness (due to anemia)
• Bruising easily
• Dental enamel defects and discoloration
• Depression
• Fatigue
• Growth delay in children
• Hair loss
• Hypoglycemia (low blood sugar)
• Irritability and behavioral changes
• Malnutrition
• Mouth ulcers
• Muscle cramps
• Nosebleed
• Seizures
• Unexplained short stature
• Skin disorders (dermatitis herpetiformis)
• Swelling, general or abdominal
• Vitamin or mineral deficiency, single or multiple nutrient (for example, iron, folate, vitamin K)

Causes:

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

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 are affected more commonly than men.

Risk Factors:

Celiac disease occurs in 5 - 15% of the children and siblings of a person with celiac disease. The disease can be triggered for the first time after surgery, viral infection, severe emotional stress, pregnancy, or childbirth.

Diagnosis:

People with celiac disease have abnormally high amounts of auto-antibodies. (Antibodies are proteins produced by the immune system that attack harmful substances in the body. Auto-antibodies, on the other hand, attack the body's own healthy tissues.)

Therefore, several antibody blood tests are useful in diagnosing celiac sprue disease.

• Immunoglobulin A (IgA)
• Anti-tissue transglutaminase (tTGA)
• IgA anti-endomysium antibodies (AEA)

Other tests that may be done include:

• An EGD (endoscopy) with small bowel biopsy may show some flattening of the villi in the intestinal lining.
• 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.
• Mildly elevated liver enzymes and abnormal blood clotting may also be noted.

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.

Preventive Care:

Since researchers are unsure of the exact cause of celiac disease, there is no known way to prevent the disease. However, being aware of your risk factors (such as a family member with the disorder) may increase the chance of early diagnosis and treatment.

Routine screening for celiac disease in people without symptoms is not done in the United States. Antibody screening tests may not be reliable in young children.

Treatment:

A life-long gluten-free diet is required. This allows the intestinal villi to heal.

Completely eliminate foods, beverages, and medications that contain wheat, barley, rye, and possibly oats.

Read food and medication labels carefully to look for "hidden" sources of these grains and their by-products. 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 begin the gluten-free diet before being diagnosed with celiac disease. Doing so will alter future testing for the disease.

To correct nutritional deficiencies, doctors 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 for achieving wellness and eliminating symptoms. Several studies have also found that sticking to such a diet helps reduce the risk of intestinal lymphoma.

Upon diagnosis, seek consultation from a registered dietitian with a specialty in celiac disease and the gluten-free diet. Joining a local and national support group can also be invaluable in helping one to cope with the disease and diet. See also: Nutrition.

Prognosis/Possible Complications:

Removing all damaging grains ("triggers" to the immune system) from the diet is the most important measure one can take to become healthy. One who follows the diet strictly can expect to lead a long, healthy life (as long as permanent damage did not occur before diagnosis).

Depending on the degree of intestinal damage prior to diagnosis, the improvement in symptoms may vary in duration once treatment begins. The gluten-free diet must be carefully and continuously followed. When untreated, the disease can cause life-threatening complications. A delayed diagnosis or failure to follow the diet places one at risk for developing other conditions.

There are numerous diseases and conditions associated with celiac disease. Some of these include:

• Anemia
• Lactose intolerance
• Dermatitis herpetiformis (a burning, itching, blistering rash)
• Type 1 diabetes
• Thyroid disease
• Downs syndrome
• Unexplained infertility
• Miscarriage
• Osteoporosis/osteopenia
• Intestinal cancer (Intestinal lymphoma is up to 40 times more common in patients with celiac disease than in those without the disease.)
• Rheumatoid arthritis
• Systemic lupus erythematosus

Other Considerations:

Since nutrients may not be absorbed properly, miscarriage, and birth defects such as neural tube defects may occur in untreated pregnant women with celiac disease.

Alternative Names:

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