Alternate Names: Sprue; Nontropical sprue; Gluten intolerance

Causes and Risks:

The exact cause of celiac disease is unknown. There is evidence that the disorder is inherited. (Its incidence is much higher in siblings.) 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) which normally absorb nutrients. Celiac disease causes the villi to become flattened and lose the ability to absorb nutrients. Weight loss, anemia, and vitamin deficiences may occur as a result of the malabsorption (inadequate absorption of nutrients from the intestinal tract).

Symptoms appear in babies within 6 months of introducing food containing gluten in the diet. The disease also affects children and adults. As many as 1 in 250 people may have the disease. (Some studies estimate that it is even more common, at 1 in 150.) Most people with the disease are undiagnosed (About 1 in 2500 are diagnosed).

Risk factors are a genetic or familial tendency toward the disease. The disorder is most common in Caucasians or northern and southern European ancestry, and 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.

Prevention: Because the exact cause is unknown, there is no way known 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.

Symptoms: IN INFANTS AND CHILDREN

• Abnormal stools
• Diarrhea
• Growth, slow (child 0 to 5 years old)
• Irritability
• Stools, bloody
• Stools, clay colored
• Vomiting
• Weight loss
• Decreased appetite (anorexia)
• Stools - foul smelling
• Muscles in arms and legs are thin and wasted
• Abdominal distention

• Abnormal appearance of the teeth
• Abdominal distention
• Abdominal pain
• Bloating
• Bone pain
• Bone tenderness
• Breathlessness (due to anemia)
• Depression
• Diarrhea
• Fatigue
• Irritability
• Muscle cramps
• Tiredness
• Vomiting
• Weight loss

• Nosebleed - symptom
• Swelling, overall
• Stools - floating

Signs and Tests: Typically, most persons with celiac disease will have symptoms of malabsorption. 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, stools may also be foul smelling and unusual colored) is often present.

Dental examination may show changes in the teeth. In fact, some cases of celiac disease are suspected by the 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. 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 can sometimes be helped by joining a support group where members share common experiences and problems. See celiac disease - support group.

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, miscarriage, and other disorders. People with celiac disease may be at a greater risk for lymphoma and intestinal cancers.

Call for an appointment with 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. Many communities have support groups such as the Celiac Disease Foundation; these groups are also a good source of information about dietary guidelines.