A 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.

Causes, incidence, and risk factors:

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 deficiencies 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 approximately 1 out of 25,000 people. Risk factors are a genetic or familial tendency toward the disease. The disorder is most common in Caucasians of 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

IN ADULTS

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

Additional symptoms that may be associated with this disease:

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.

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

Last updated May 21, 1999

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