Celiac disease

Celiac disease (also called sprue, nontropical sprue, celiac sprue, and gluten sensitive enteropathy) occurs when the body reacts abnormally to gluten, aprotein found in wheat, rye, barley, and oats. Gluten causes an inflammatoryresponse in the small intestine, which damages the tissues and results in impaired ability to absorb nutrients from foods. The inflammation and malabsorption create wide-ranging problems in many systems of the body.

The exact cause of celiac disease is not clearly understood, but it is knownthat both heredity and the immune system play a part. When food containing gluten reaches the small intestine, the immune system begins to attack a substance called gliadin, which is found in the gluten. The resulting inflammationcauses damage to the delicate finger-like structures in the small intestine,called villi, where food absorption actually takes place.

The most commonly recognized symptoms of celiac disease relate to the improper absorption of food in the gastrointestinal system. The patient will have diarrhea and fatty, greasy, unusually foul-smelling stools. The patient may complain of excessive gas (flatulence), distended abdomen, weight loss, and generalized weakness. A distinctive skin rash, called dermatitis herpetiformis, is present in approximately 10% of patients with celiac disease.

Unrecognized and therefore untreated celiac disease may cause or contribute to a variety of other medical conditions. The decreased ability to digest, absorb, and utilize food properly (malabsorption) may cause anemia from iron deficiency or easy bruising from a lack of vitamin K. Poor mineral absorption may result in osteoporosis, or "brittle bones," which may lead to bone fractures. Vitamin D levels may be insufficient and bring about a "softening" of bones (osteomalacia), which produces pain and bony deformities, such as flattening or bending. Defects in the tooth enamel, characteristic of celiac disease,may be recognized by dentists. Celiac disease may cause a failure to thrive in infants, or lack of proper growth in children and adolescents. People withceliac disease may also experience lactose intolerance.

Many disorders are associated with celiac disease, though the nature of the connection is unclear. One type of epilepsy is linked to celiac disease. Oncetheir celiac disease is successfully treated, a significant number of these patients have fewer or no seizures. Patients with alopecia areata, a conditionwhere hair loss occurs in sharply defined areas, have been shown to have a higher risk of celiac disease than the general population. There appears to bea higher percentage of celiac disease among people with Down syndrome, but the link between the conditions is unknown.

People with insulin dependent diabetes (type I) have a much higher incidenceof celiac disease. Patients with other conditions where celiac disease may bemore commonly found include those with juvenile chronic arthritis, some thyroid diseases, and IgA deficiency.

Because of the variety of ways celiac disease can manifest itself, it is often not discovered promptly. The condition may persist without diagnosis for solong that the patient accepts a general feeling of illness as normal. This leads to further delay in identifying and treating the disorder.

If celiac disease is suspected, a blood test can be ordered that looks for the antibodies that the immune system produces in celiac disease. An abnormal result points towards celiac disease, but further tests are needed to confirmthe diagnosis. Doctors may order a test of iron levels in the blood because low levels of iron (anemia) may accompany celiac disease. Doctors may also order a test for fat in the stool, since celiac disease prevents the body from absorbing fat from food.

The next step is a biopsy of the small intestine. This is usually done by a gastroenterologist, a physician who specializes in diagnosing and treating bowel disorders. It is generally performed in the office, or in an outpatient department in a hospital. The patient remains awake, but is sedated. A narrow tube is passed through the mouth, down through the stomach, and into the smallintestine. A small sample of tissue is taken and sent to the laboratory foranalysis. If it shows a pattern of tissue damage characteristic of celiac disease, the diagnosis is established.

The patient is then placed on a gluten-free diet (GFD). Gluten is present inany product that contains wheat, rye, barley, or oats. In addition to the many obvious places gluten can be found in a normal diet, such as breads, cereals, and pasta, there are many hidden sources of gluten. These include ingredients added to foods to improve texture or enhance flavor and products used infood packaging. Gluten may even be present on surfaces used for food preparation or cooking.

Fresh foods that have not been artificially processed, such as fruits, vegetables, and meats, are permitted as part of a GFD. Gluten-free foods can be found in health food stores and in some supermarkets.

Treating celiac disease with a GFD is almost always completely effective. Once the diet has been followed for several years, individuals with celiac disease have similar mortality rates as the general population. Gastrointestinal complaints and other symptoms are alleviated. People who have experienced lactose intolerance related to their celiac disease usually see those symptoms subside. Secondary complications, such as anemia and osteoporosis, resolve in almost all patients, as well. However, about 10% of people with celiac diseasedevelop a cancer involving the lymphatic system (lymphoma).

Experts emphasize the need for lifelong adherence to the GFD to avoid the long-term complications of this disorder. They point out that although the disease may have symptom-free periods if the diet is not followed, silent damage continues to occur. Celiac disease cannot be "outgrown" or cured, according tomedical authorities.

There are a small number of patients who develop a refractory type of celiacdisease, where the GFD no longer seems effective. Once the diet has been thoroughly assessed to ensure no hidden sources of gluten are causing the problem, medications may be prescribed. Steroids or immunosuppressant drugs are often used to try to control the disease. It is unclear whether these efforts meet with much success.

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