Celiac Sprue: Overview and Risk Factors

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.