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Celiac disease - sprue

Celiac disease is a condition that creates inflammation in the small intestine, and damage in the lining. This prevents important components of food from being absorbed. The damage to the lining of the intestine comes from a reaction to eating gluten, which is found in wheat, rye, barley, and possibly oats, and in food made from these ingredients.

Alternative Names

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

Causes

The exact cause of celiac disease is unknown. The lining of the intestines have small projections contains areas called villi. These structures help absorb nutrients. When people with celiac disease eat foods or use products that contain gluten, their immune system reacts by damaging the villi. As a result, the villi are unable absorb nutrients properly. Therefore, a person becomes malnourished , no matter how much food he or she eats.

The disease can develop at any point in life, from infancy to late adulthood.

People who have a family member with celiac disease are at greater risk for developing the disease. The disorder is most common in Caucasians and persons of European ancestry. Women are affected more often than men.

People with celiac disease are more likely to have:

Symptoms

The symptoms of celiac disease can be different from person to person. This can make diagnosis difficult. For example, one person may have constipation, a second may have diarrhea, and a third may have no problem with stools.

Gastrointestinal symptoms include:

Other problems that can develop over time because the intestines do not absorb key nutrients include:

  • Easy bruising
  • Depression or anxiety
  • Fatigue
  • Growth delay in children
  • Hair loss
  • Itchy skin with a rash (dermatitis herpetiformis )
  • Missed menstrual periods
  • Mouth ulcers
  • Muscle cramps and joint pain
  • Nosebleeds
  • Seizures
  • Tingling or numbness in the hands or feet
  • Unexplained short height

Children with celiac disease may have:

  • Defects in the tooth enamel and changes in tooth color
  • Delayed puberty
  • Diarrhea, constipation, fatty or foul-smelling stools, nausea, or vomiting
  • Irritable and fussy behavior
  • Poor weight gain
  • Slowed growth and shorter than normal height for their age

Exams and Tests


  • Bone density
  • Cholesterol (may be low)
  • Complete blood count (CBC - test for anemia)
  • Comprehensive metabolic panel
  • Folate level (serum)
  • Iron level (serum)
  • Prothrombin time
  • Vitamin B12 level (serum)
  • Vitamin D level

Blood tests can detect antibodies, called antitissue transglutaminase antibodies (tTGA) or anti-endomysial antibodies (EMA) which may help detect the condition. The health care provider will order these antibody tests if celiac disease is suspected.

If the tests are positive, upper endoscopy is often performed to sample a piece of tissue (biopsy) from the first part of the small intestine (duodenum). The biopsy may show a flattening of the villi in the parts of the intestine below the duodenum.

Genetic testing of the blood can also be done to help determine who may be at risk for celiac disease.

A follow-up biopsy or blood test may be ordered several months after the diagnosis and treatment. These tests assess how well treatment is working. Normal results mean that you have responded to treatment. This confirms the diagnosis. However, this does not mean that the disease has been cured.

Treatment

Celiac disease cannot be cured. Your symptoms will go away and the villi in the lining of the intestines will heal if you follow a lifelong gluten-free diet . Do not eat foods and beverages or take medicines that contain wheat, barley, rye, and possibly oats.

You must read food and drug labels carefully to look ingredients that may include these grains. It may be hard to stick to a gluten-free diet because wheat and barley grains are common in the American diet. Over time, most people are able to adapt and get better. You should NOT begin the gluten-free diet before you are diagnosed. Starting the diet will affect testing for the disease.

Your health care provider may prescribe vitamin and mineral supplements to correct nutritional deficiencies. Sometimes, short-term use of corticosteroids (such as prednisone) may be needed if sprue does not respond to treatment. In most cases, following a well-balanced, gluten-free diet is the only treatment you need to stay well.

When you are diagnosed, get help from a registered dietitian who specializes in celiac disease and the gluten-free diet. A support group may also help you cope with the disease and diet.

Outlook (Prognosis)

Following a gluten-free diet heals the damage to the intestines and prevents further damage. This healing most often occurs within 3 - 6 months in children. Recovery may take 2 - 3 years in adults.

Rarely, long-term damage will be done to the lining of the intestines before the diagnosis is made.

Some problems caused by celiac disease may not improve, such as a shorter than expected height and damage to the teeth.

Possible Complications

You must carefully continue to follow the gluten-free diet. When untreated, the disease can cause life-threatening complications.

Delaying diagnosis or not following the diet puts you at risk for related conditions such as:

When to Contact a Medical Professional

Call your health care provider if you have symptoms of celiac disease.

Prevention

Because the exact cause is unknown, there is no known way to prevent the development of celiac disease. However, being aware of the risk factors (such as having a family member with the disorder) may increase your chances of early diagnosis, treatment, and a long, healthy life.

References

Green PH, Cellier C. Celiac disease. N Engl J Med. 2007;357:1731-1743.

Kagnoff MF. AGA Institute medical position statement on the diagnosis and management of celiac disease. Gastroenterology. 2006;131(6): 1977-1980.

Semrad CE. Approach to the patient with diarrhea and malabsorption. In: Goldman L, Shafer AI, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 142.

Updated: 2/21/2014

Todd Eisner, MD, Private practice specializing in Gastroenterology, Boca Raton, FL. Affiliate Assistant Professor, Florida Atlantic University School of Medicine. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.


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