Sprue

Related Terms

Antibodies, antibody, autoantibodies, autoantibody, autoimmune, autoimmune disease, autoimmune disorder, autoimmunity, biopsy, celiac, coeliac disease, cornmeal, digestive tract, endoscope, endoscopy, GFD, gluten, gluten-free, gluten-free diet, immune, immune defense system, immune reaction, immune response, immune system, intestinal lining, intestine, malabsorption, nutrient deficiency, potato flour, rice flour, vitamin deficiency, wheat.

Background

Celiac disease, also called celiac sprue, is a digestive disorder that occurs when an individual's immune system overreacts to the protein gluten, or other proteins within gluten such as gliadin, found in grains including wheat, rye, barley, and to some degree, oats. When a patient with the disease eats food that contains gluten, the immune system's response damages the intestinal lining. This causes symptoms of abdominal pain and bloating after consuming gluten.
Additionally, complications, including poor absorption, may occur if the patient continues to eat gluten-containing foods. When the intestinal lining is damaged, patients have difficulty absorbing nutrients.
It has not been determined what exactly triggers this reaction in celiac patients. However, celiac disease is associated with autoimmune disorders, such as lupus. Autoimmune disorders occur when the patient's immune system mistakenly identifies body cells as harmful invaders, such as bacteria. As a result, the immune cells in celiac patients attack the patient's intestinal cells.
Researchers at the National Digestive Diseases Information Clearinghouse (NDDIC) estimate that one out of 133 people in the United States have celiac disease. Prevalence is even higher, one out of 22 people, among patients who have immediate family members (parent or sibling) who have the disease. This suggests that the disease may be inherited (passed down through families) in some cases.
Individuals can develop this disease at any age, but it is most commonly diagnosed in patients who are eight to 12 months old or in patients ages 30-40.
Although there is currently no known cure for celiac disease, the condition can be managed with a gluten-free diet. In general, patients who strictly follow a gluten-free diet can expect to live normal, healthy lives. Symptoms usually subside in several weeks and patients will be able to absorb food normally once they avoid eating gluten. A dietician or certified nutritionist may help a patient with celiac disease develop a healthy diet. Patients with celiac disease may also find gluten-free cookbooks to be a helpful resource. Many products, including rice flour and potato flour, can be used as substitutes for gluten.

Diagnosis

Blood tests: If celiac disease is suspected, blood tests will be performed to determine whether or not the patient has autoantibodies associated with the disease. Specific autoantibodies in the blood bind to the patient's own body cells. This triggers other cells to attack and destroy cells in the intestinal lining. If these autoantibodies are present in a patient's blood, a positive diagnosis is made.
Blood tests may also be performed to determine whether or not the patient has antibodies to gluten. During this procedure, a sample of the patient's blood is combined with gluten to determine whether or not antibodies are present. If antibodies are present, it suggests the patient may have celiac disease.
Biopsy: An intestinal biopsy may be performed to confirm a diagnosis, but it is not often required. This procedure is more invasive and expensive than a blood test. During the procedure, a thin, flexible tube called an endoscope is inserted through the patient's mouth and into the intestine. A small tissue sample is taken and analyzed under a microscope. If the intestinal tissue is damaged, a positive diagnosis is made.

Complications

Nutritional deficiencies: When a patient with celiac disease consumes gluten, the immune cells attack enzymes in the intestine that are needed for digestion and absorption of nutrients. Therefore, nutritional deficiencies are a complication of untreated celiac disease. Children may experience stunted growth if they are not receiving enough nourishment.
Patients who continue to eat gluten despite their condition may develop anemia (low levels of iron in the blood) because they are unable to absorb iron, folic acid, or vitamin B12.
Patients who continue to eat gluten often have low levels of vitamin K and D because they are normally absorbed in the intestine. Vitamin K is needed for the production of blood clotting factors. Patients with vitamin K deficiency have an increased risk of bleeding. Patients with vitamin D deficiency may have low levels of calcium in the blood, which may lead to bone disorders such as osteoporosis (hollow, brittle bones).
If left untreated, poor nutrition can potentially be fatal.
Other disorders: Celiac disease is associated with autoimmune disorders. The most common autoimmune disorders associated with the disease are lupus erythematosus, type I diabetes, rheumatoid arthritis, thyroid disease, and microscopic colitis (inflammation of the colon). The prognosis for patients with co-existing autoimmune disorders depends on the specific disorder. Most autoimmune disorders are chronic. However, many disorders can be managed with treatment such as immunosuppressants and anti-inflammatories.

Treatment

General: Although there is currently no cure for celiac disease, the condition can be managed with diet. Symptoms will subside within several weeks and patients will be able to absorb food normally once they avoid eating gluten. However, it may take several months in children and two to three years in elderly patients for the intestine to fully recover.
Support: Healthcare providers may recommend a dietitian or nutritionist who can help a patient plan an appropriate gluten-free diet. These professionals can also help patients determine whether or not supplementation with vitamins and minerals is necessary.
Gluten-free diet: Patients should avoid all foods that contain gluten. This includes any type of wheat (including farina, graham flour, semolina, and durum), barley, rye, bulgur, Kamut, kasha, matzo meal, spelt, and triticale. Therefore, foods such as bread, cereal, crackers, pasta, cookies, cake, pie, gravy, and sauce should be avoided unless they are labeled as gluten-free.
Many less obvious foods contain gluten. For instance, grains that contain gluten are often used in food additives, such as modified food starch and malt flavoring. Also, some medications, herbal supplements, and vitamins may contain gluten as a binding agent. Lipstick and postage stamps may contain gluten.
Studies have shown that a gluten-free diet may improve iron deficiency (anemia), malnutrition, anxiety, and depression. Some reports exist of mood swings or depression after adopting a gluten free diet, purportedly due to issues relating to dietary adherence. However, the overwhelming body of scientific evidence concludes that a gluten-free diet will reduce these symptoms.
How to avoid gluten: Patients should carefully read the labels of their foods. To help patients identify foods like gluten, the U.S. Congress passed the Food Allergen Labeling and Consumer Protection Act of 2004 (FALCPA). The law, which went into effect January 1, 2006, requires food manufacturers to clearly state on their packages whether the food is made with any ingredients that contain products derived from milk, eggs, fish, crustacean shellfish, tree nuts, peanuts, wheat, or soybeans. This act also requires the FDA to develop and finalize rules for the use of the term "gluten-free" on product labels by August 2008.
Food products can become cross-contaminated with gluten if they come into contact with gluten. For instance, a knife that was used to cut bread should be washed thoroughly with soap and water before it is used to cut food for a patient with celiac disease. Cross contamination may also occur if bread and vegetables were cut on the same cutting board.
Hidden gluten can be found in some unlikely foods, such as cold cuts, soups, hard candies, soy sauce, and many low or non-fat products (such as licorice and jelly beans). Gluten may also come in forms such as vegetable proteins and starch, modified food starch (when derived from wheat instead of corn), maltodextrin, malt flavoring, and glucose syrup. Many common ingredients contain wheat or barley derivatives.
Patients with celiac disease should always ask about the ingredients in food when dining at a restaurant or someone else's home.
Patients should consult their healthcare providers and pharmacists before taking any drugs, herbs, or supplements because they may contain gluten.
Read the labels of cosmetics because some beauty products, such as lipstick, may contain gluten.
Use self-adhesive postage stamps because stamps that require moistening may be contaminated with gluten.
Foods that are safe to eat: Today, patients can choose from a wide variety of gluten-free products at grocery stores. There are gluten-free substitutes for many foods and beverages, including cookies and beer. If gluten-free foods are not readily available at the local supermarket, they can be purchased online.
Patients can eat fresh meats, fish, and poultry that are not breaded or marinated. Most dairy products are safe to eat, as well as fruits, vegetables, rice, potatoes, and gluten-free flours that are made from rice, soy, potato, or corn.
Although amaranth, buckwheat, and quinoa are gluten-free when they are grown, they may become contaminated with other grains during harvesting and/or processing. Even though oats may not be harmful for most patients with celiac disease, they are often contaminated with wheat. Therefore, oats should be avoided as a precautionary measure.
Cooking: Patients with celiac disease do not necessarily have to give up their favorite foods because they cannot eat gluten. Substitutes for wheat flour can be used for many recipes. For instance, if a recipe calls for one teaspoon of wheat flour, patients can use 1.5 teaspoon of potato starch, cornstarch, rice flour, or arrowroot starch instead. One cup of wheat flour may be substituted with one cup of fine cornmeal, 3/4 cup of coarse cornmeal, 3/4 cup of rice flour, or 5/8 cup of potato flour. Gluten-free cookbooks are widely available to help patients maintain their quality of life with respect to food.

Integrative therapies

Note: There is currently a lack of available scientific evidence on the use of integrative therapies for the treatment or prevention of celiac disease. The therapies listed below should be used only under the supervision of a qualified healthcare provider, and should not be used in replacement of other proven therapies or preventive measures.
Unclear or conflicting scientific evidence:
Zinc: In limited early research, oral zinc supplements did not seem to improve the clinical condition of patients with unresponsive celiac syndrome. More research is needed in this area.
Zinc is generally considered safe when taken at the recommended dosages. Use amounts regularly exceeding the recommended upper tolerance levels (greater than 40 milligrams daily) under a physician's guidance only. Use cautiously in patients with bleeding disorders, diabetes, or low blood sugar levels, or in patients taking agents for these conditions. Use cautiously in patients with high cholesterol or blood fats, a high risk of developing heart disease, various skin disorders, gastrointestinal disorders, liver disease, genitourinary conditions, blood disorders, neurological disorders, pulmonary or respiratory disorders, immune disorders, or kidney disease, or in patients taking antidepressants, potassium-sparing diuretics, antibiotics (particularly tetracyclines and quinolones), iron, penicillamine, thyroid hormones, or copper. Avoid in patients who are homozygous for hemochromatosis (a metabolic disorder involving the deposition of iron-containing pigments in the tissues and characterized by bronzing of the skin, diabetes, and weakness) or with a known allergy or hypersensitivity to zinc compounds. Avoid use of intranasal Zicam?.
Traditional or theoretical uses that lack sufficient evidence:
Buckwheat: Buckwheat (Fagopyrum esculentum) has been suggested as acceptable for, or tolerated by, people with celiac disease. However, there is currently a lack of high-quality human studies in this area.
Use cautiously in patients with celiac disease, diabetes, or hypoglycemia. Use cautiously in those using agents that lower blood glucose, cholesterol, or blood pressure, or that increase sensitivity to light. Avoid nonfood parts of the buckwheat plant in pregnant women. Avoid with known allergy or hypersensitivity to buckwheat, its constituents, or members of the Polygonaceae family.
Carob: Germ proteins in carob (Ceratonia siliqua) have been found to have similar properties as wheat gluten and have been used in gluten-free baked foods. Carob germ flour has been used as an alternative to wheat flour in gluten-free bread. However, there is currently a lack of human studies on the safety and efficacy of carob for celiac disease.
Use cautiously in patients with anemia, known allergy to peanuts and other nuts, complications with powdered, bulk forming laxative drinks, diabetes, or high cholesterol. Use cautiously if taking oral herbs or drugs. Use cautiously in hypouricemic patients. Avoid if allergic/hypersensitive to carob (Ceratonia siliqua), its constituents, or any plants in the Fabaceae family, including tamarind. Avoid with metabolic disorders, with a chromium, cobalt, copper, iron, or zinc disorder or deficiency, renal disorders, or acute diarrhea. Avoid in underweight infants. Avoid if pregnant or breastfeeding.
Cassava: Cassava (Manihot esculenta), also known as manioc or yucca, is a starchy root vegetable that is widely grown and consumed as a staple food source in tropical countries. However, potentially toxic levels of cyanic glycosides are naturally present in cassava, and, if not removed during processing, they are converted to hydrogen cyanide, which may cause a number of diseases, and in some cases death, particularly in the presence of protein-deficient diets. The world market for cassava is limited, due to the abundance of substitutes; however, it may be increasing in popularity as a wheat-free alternative for individuals with celiac disease.
Cassava is thought to be safe when taken in small amounts for short periods of time by people who are not malnourished. Cassava is thought to be safe in pregnant and breastfeeding women who are not allergic and who take it in amounts that are generally found in foods. However, levels higher than those normally found in the diet are not suggested due to toxins that may cause thyroid and brain defects in babies. Use cautiously when the cassava contains 50-100 parts per million of cyanogenic content and is taken for extended periods of time by otherwise healthy adults. Use cautiously in people who are taking birth control or chondroitin. Avoid if allergic or sensitive to cassava, its parts, or members of the Euphorbiaceae family. Avoid if allergic or sensitive to latex or to yucca. Avoid using when the amount of toxins in the cassava product cannot be determined. High levels of toxins in cassava may cause disease and death. Avoid in people who have iodine deficiency, malnutrition, or thyroid deficiency, or those who are eating a low-protein diet.
Folic acid: The role of folic acid status in people with celiac disease has been investigated in preliminary research. High-quality human studies are needed in this area.
Use cautiously if receiving coronary stents and with anemia and seizure disorders. It is suggested that pregnant women consume 400 micrograms daily in order to reduce the risk of the fetus developing a defect. Folate is likely safe if breastfeeding. Avoid if allergic or hypersensitive to folate or any folate product ingredients.
Iron: The role of iron deficiency in people with celiac disease has been investigated in preliminary research. High-quality human studies are needed in this area.
Use iron supplements cautiously in patients with gastrointestinal disorders, kidney disease, intestinal disease, peptic ulcer disease, enteritis, colitis, pancreatitis, hepatitis, or alcoholism; in those who plan to become pregnant; in uremic patients treated with dialysis; or in patients over age 55 with a family history of heart disease. Use cautiously during pregnancy for elevating iron stores in bone marrow. Pregnant or breastfeeding women should consult a healthcare professional before beginning iron supplementation. Avoid iron in patients with or at risk for iron overload, due to hematochromatosis (a disease in which too much iron is absorbed from the gastrointestinal tract, often genetic) or any other cause. Avoid in patients with hemolytic anemia. Avoid with known allergy/hypersensitivity to products containing iron. Contact hypersensitivity to iron has been reported.
Pancreatin: In early research, pancreatic enzymes, such as pancreatin, have been investigated as possible treatments for celiac disease. A conclusion on the safety or effectiveness of this use cannot be determined at this time.
Pancreatin supplements should be used only on the advice of a medical practitioner. Pancreatin is likely safe when taken by mouth in doses recommended by a healthcare practitioner for patients with exocrine pancreatic insufficiency, a condition in which food is not properly digested because the pancreas does not make an adequate amount of digestive enzymes. Use with caution in patients with diabetes or hypoglycemia, or in those taking drugs, herbs, or supplements that affect blood sugar. Use with caution in patients at risk for developing headaches or with a history of migraines, or in patients with gastrointestinal, lung, respiratory, or kidney disorders. Use with caution in patients using calcium carbonate- or magnesium hydroxide-containing antacids, folic acid, or iron. Avoid in patients with allergy or hypersensitivity to pancreatin, pork protein, or any other components of pancreatin. Avoid in pregnant or breastfeeding women.
Quinoa: Quinoa (Chenopodium quinoa) has been used as a substitute for wheat, especially by those on a gluten-free diet due to celiac disease or other conditions. However, high-quality human research is lacking.
Avoid if allergic or hypersensitive to quinoa, its constituents, or members of the Amaranthaceae family. Use unwashed quinoa cautiously because it is naturally coated with saponins. Avoid if pregnant or breastfeeding.
Selenium: The role of selenium deficiency in people with celiac disease has been investigated in preliminary research. High-quality human studies are needed in this area.
Use cautiously at doses from 27-2,310 milligrams daily, as well as in patients on hemodialysis, in patients with iodine deficiency, high cholesterol, or immune disorders, and in those using immunosuppressants, corticosteroids, antacids, erythropoietin (EPO), cholesterol-lowering medications, oral contraceptives, astragalus, or iron supplements. Avoid in patients at high risk of nonmelanoma skin cancers or at risk for developing diabetes. Avoid in patients with known allergy or hypersensitivity to products containing selenium; however, selenium is a trace element, and hypersensitivity is unlikely.
Vitamin E: The role of vitamin E deficiency in people with celiac disease has been investigated in preliminary research. High-quality human studies are needed in this area.
Use long-term vitamin E cautiously. Use cautiously in patients with heart conditions, skin conditions, kidney impairment, Alzheimer's disease, cognitive decline, retinitis pigmentosa (loss of peripheral vision), or in patients who smoke, or in preterm infants. Avoid high doses of vitamin E. Avoid above the recommended daily level in pregnant women and breastfeeding women. Avoid in patients with bleeding disorders or in those taking drugs that may increase the risk of bleeding. Avoid if allergic or hypersensitive to vitamin E.
Vitamin K: The role of vitamin K levels in people with celiac disease has been investigated. Although vitamin K deficiency is rare, patients who have celiac disease have an increased risk of developing this condition. High-quality human studies are needed in this area.
Conditions that interfere with absorption of vitamin K may lead to deficiency, including short bowel syndrome, cystic fibrosis, malabsorption (various causes), pancreas or gallbladder disease, persistent diarrhea, sprue, and ulcerative colitis. Use cautiously in patients with skin or gastrointestinal conditions, in pregnant women, and in those taking lipid-lowering drugs. Injection into the muscle or vein should only be done by a healthcare professional. Many serious side effects have occurred after injection. Avoid in patients with known allergy or hypersensitivity to vitamin K. Avoid supplemental vitamin K in patients using warfarin or other blood-thinning agents unless otherwise directed by a healthcare professional. Avoid use of vitamin K3 supplements or menadiol.

Prevention

Currently, there is no known method to prevent celiac disease.
Patients who have celiac disease can prevent symptoms from returning by adhering to a gluten-free diet. A certified dietitian can help patients plan appropriate and healthy gluten-free diets.
Patients should carefully read the labels of all food products. Food manufacturers in the United States are required to clearly state whether their products contain wheat.
If children have celiac disease, their baby-sitters, teachers, and other caretakers should be informed of their conditions.
Patients with celiac disease should always ask about ingredients in the food when dining at a restaurant or someone else's home.
Patients should consult their healthcare providers and pharmacists before taking any drugs, herbs, or supplements because they may contain gluten.
Read the labels of cosmetics because some beauty products, such as lipstick, may contain gluten.
Use self-adhesive postage stamps because stamps that require moistening may be contaminated with gluten.
Food products can become cross-contaminated with gluten if they come into contact with gluten. For instance, a knife that was used to cut bread should be washed thoroughly with soap and water before it is used to cut food for a patient with celiac disease. Cross contamination may also occur if bread and vegetables were cut on the same cutting board.

Author information

This information has been edited and peer-reviewed by contributors to the Natural Standard Research Collaboration (www.naturalstandard.com).

Bibliography

Bardella MT, Velio P, Cesana BM, et al. Coeliac disease: a histological follow-up study. Histopathology. 2007 Mar;50(4):465-71. .
Catassi C, Kryszak D, Louis-Jacques O, et al. Detection of Celiac Disease in Primary Care: A Multicenter Case-Finding Study in North America. Am J Gastroenterol. 2007 Mar 13; [Epub ahead of print]. .
Celiac Disease Foundation. .
Garsed K, Scott BB. Can oats be taken in a gluten-free diet? A systematic review. Scand J Gastroenterol. 2007 Feb;42(2):171-8. .
Marmouz F. Adult coeliac disease. Allerg Immunol (Paris). 2007 Jan;39(1):23-5. .
National Digestive Diseases Information clearinghouse (NDDIC). .
Natural Standard: The Authority on Integrative Medicine. .
No authors listed. NIH Consensus Development Conference on Celiac Disease. NIH Consens State Sci Statements. 2004 Jun 28-30;21(1):1-23. .
U.S. Food and Drug Administration (FDA). .

Causes

General: When a celiac patient eats gluten, or other protein components of gluten, such as gliadin, the body's immune system overreacts. Gluten is present in all types of wheat (including farina, graham flour, semolina, and durum), barley, rye, bulgur, Kamut, kasha, matzo meal, spelt, and triticale. The gluten is mistaken for a harmful invader, such as bacteria, and an attack is launched. Immune system cells flood to the stomach and intestine to destroy the gluten. However, among these immune cells are autoantibodies that attack the lining of the intestine by mistake. As a result, the intestinal lining becomes damaged.
Gliadin is a protein component of gluten, found in wheat and several other cereal grains of the genus Triticum. Patients with celiac disease are sensitive to the ?, ?, and ? forms of gliadins. In response to gliadin, anti-gliadin IgA antibodies are produced, which are reportedly found in many patients with celiac disease.
Inheritance: Researchers believe that many cases of celiac disease are inherited (passed down through families). Researchers estimate that if someone in a patient's immediate family (parent or sibling) has celiac disease, the patient has a 5-15% chance of developing the disease as well.
Trauma: It also appears that many cases of celiac disease develop after trauma, such as an infection, stress, physical injury, surgery, or pregnancy.
Other disorders: Celiac disease is associated with autoimmune disorders. Autoimmune disorders occur when the immune system attacks the body by mistake. Autoantibodies in the blood bind to components of an individual's own cells, triggering other cells to attack the body. The most common autoimmune disorders associated with celiac disease are lupus erythematosus, type I diabetes, rheumatoid arthritis, thyroid disease, and microscopic colitis (disorder that causes inflammation of the colon).

Symptoms

In general, patients who follow a gluten-free diet can expect to live normal, healthy lives. Most patients with celiac disease experience nonspecific symptoms after consuming gluten, including intermittent diarrhea, abdominal pain, and bloating. Some patients may experience no symptoms.
If a patient is not absorbing enough nutrients, symptoms may include weight loss, diarrhea, abdominal cramps, gas, bloating, fatigue, foul-smelling or grayish stools that may be oily, stunted growth in children, and osteoporosis (hollow, brittle bones).