Celiac sprue (also known as celiac disease, gluten-sensitive enteropathy, or nontropical sprue) is a disorder in which the immune system inappropriately attacks the lining of the small intestine.

Patients with celiac sprue are sensitive to gluten, a protein contained in wheat, barley, and rye. The gluten, which acts as an allergen, causes the body's immune system to damage the lining of the small intestine, resulting in malabsorption of fat, calcium, iron, folate, and other nutrients.

Symptoms typically first appear when wheat is introduced into a child's diet, usually at 6 to 12 months. Children may experience delayed growth, irritability, vomiting, constipation, large stools, swelling, and frequent respiratory infections.

In some cases, the disease may not appear until later in life, typically between the ages of 10 and 40. Most adults do not have noticeable symptoms. In some cases, patients may experience diarrhea, weight loss, abdominal swelling, and bloating.

Over time, malabsorption of vitamin D and calcium may result in rickets, osteoporosis, and bone fractures. Iron malabsorption can result in anemia. For reasons that are not entirely clear, people with celiac sprue are at increased risk for cancers of the esophagus and small intestine, and lymphoma.

Risk Factors

• Genetics: Celiac sprue occurs in 10 percent of first-degree relatives of affected patients. More than 95 percent of affected people have specific gene mutations. 
• Immune disorders: People with a history of immune disorders are at increased risk.
• Environment: Infectious agents, including respiratory and gastrointestinal viruses, may play a role in susceptible patients.
• Type I diabetes mellitus: About 5 percent of people with type I diabetes mellitus also have celiac disease.
• Down syndrome: People with Down syndrome have double the risk of celiac disease, compared with the general population.

Celiac Sprue: Diagnosis and Treatment

Diagnosis

• The evaluation begins with a medical history and physical examination.
  
• Blood testing can be used to screen for antibodies that commonly occur in celiac disease. Blood tests may also reveal anemia.
  
• If the diagnosis is in doubt after blood testing, a biopsy of the small intestine will establish the diagnosis. Rarely, a follow-up biopsy may be performed for comparison after the patient has followed a gluten-free diet for three to six months.
  

Treatment

• The cornerstone of treatment is to avoid dietary gluten (see Nutritional Considerations).
• In patients who do not respond to gluten avoidance, treatment with steroid medications may be necessary.

Celiac Sprue: Nutritional Considerations

The following nutritional steps are key to managing celiac disease:

• A gluten-free diet
  
  Gluten-containing foods include wheat, barley, and rye, among others. Careful reading of food labels is important. Patients should consult with an experienced dietitian to identify these foods and ensure adequate consumption of essential nutrients.
  
  Although gluten is also found in oats, some studies suggest that pure oat flour can usually be tolerated. Many doctors allow celiac patients to introduce small amounts of oats into their diets once the disease is under control. Initially, patients are limited to less than 2 grams per day, building up to 40-60 grams per day). However, consumers should be aware that commercial oat products may be contaminated with other gluten-containing grains.

• Preventing nutrient deficiencies
  
  Patients who follow a gluten-free diet are at risk for deficiencies of B vitamins (including folate), calcium, vitamin D, iron, zinc, magnesium, and fiber. Further, because celiac disease damages the ability of the small intestine to absorb fat, nutritional deficiencies of fat-soluble vitamins (vitamins A, D, E, and K) may occur. Daily multiple vitamins may be helpful, and dietitians can assess whether additional dietary supplements are required.

• A consultation with a dietician is advisable to assist with dietary changes.