Celiac Disease (Gluten Enteropathy)

What is celiac disease?

Celiac disease is a disease of the diminutive intestine. The small intestine is a 22 foot long tube that begins at the stomach and ends at the enormous intestine (colon). The first 1-1/2 feet of the slight intestine (the part that is attached to the stomach) is called the duodenum, the middle fragment is called the jejunum, and the last piece (the part that is attached to the colon) is called the ileum. Food empties from the stomach into the small intestine where it is digested and absorbed into the body. While food is being digested and absorbed, it is transported by the cramped intestine to the colon. What enters the colon is primarily undigested food. In celiac disease, there is an immunological (allergic) reaction within the inner lining of the small intestine to proteins (gluten) that are present in wheat, rye, barley and, to a lesser extent, in oats. The immunological reaction causes inflammation that destroys the lining of the minute intestine. This reduces the absorption of dietary nutrients and can lead to symptoms and signs of nutritional, vitamin, and mineral deficiencies. Other names for celiac disease include sprue, nontropical sprue, gluten enteropathy, and adult celiac disease. (Tropical sprue is another disease of the small intestine that occurs in tropical climates. Although tropical sprue may cause symptoms that are similar to celiac disease, the two diseases are not related.)

Celiac disease is well-liked in European countries, particularly in Ireland, Italy, Sweden, and Austria. In Northern Ireland, for example, one in every 300 people has celiac disease. In Finland, the prevalence may be as high as one in every 100 persons. Celiac disease also occurs in North America where the prevalence has been estimated at one in every 3000 people. Unfortunately, most population studies underestimate the prevalence of celiac disease because many patients who develop celiac disease have few or no symptoms until later in life. In fact, a original study in the United States suggests that the prevalence of celiac disease in the United States is similar to Europe.

What causes celiac disease?

The destruction of the inner lining of the small intestine in celiac disease is caused by an immunological (allergic) reaction to gluten in the diet that inflames and destroys the inner lining of the small intestine. There is evidence that this reaction is partially genetic and inherited. Thus, approximately 10% of first-degree relatives (parents, siblings or children) of individuals with celiac disease also will have celiac disease. In addition, in approximately 30% of fraternal twins and 70% of identical twins, both twins will have celiac disease. Finally, sure genes have been found to be more common among individuals with celiac disease than among individuals without celiac disease.

Gluten is a family of proteins present in wheat. Some of the proteins that make up gluten (the ones that are dissolved by alcohol) are called gliadin. It is the gliadin in gluten that causes the immunological reaction in celiac disease. The mechanism whereby gliadin becomes toxic (damaging) is not clear; however, much scientific study is being done, and we are beginning to understand the mechanism.

Proteins, including gliadin, are long chains of amino acids-up to several hundred–attached to each other. Normally during digestion, digestive enzymes within the itsy-bitsy intestine break-up proteins into single amino acids and smaller chains of amino acids. This is necessary because the intestine only can absorb single amino acids or, at most, chains of 3-4 amino acids. Single amino acids and chains of several amino acids do not cause problems for the intestine. It appears, however, that gliadin is not completely broken-up by intestinal enzymes. Several longer chains of amino acids remain intact. Somehow these larger chains enter the cells lining the intestine, perhaps because the cells are abnormally permeable (leaky) to longer chains of amino acids. Some of these longer chains are toxic (damaging) to the intestinal cells. One of the longer chains attaches to an enzyme within the cells, tissue transglutaminase. In individuals with celiac disease, the complex of the longer chain of amino acids and tissue tranglutaminase sets off an immune reaction that attacks the complex and at the same time damages the intestinal cells.

Barley and rye contain gliadin-like proteins and can cause celiac disease in genetically-predisposed individuals. Oats also occupy gliadin-like proteins, but unlike barley and rye, the gliadin-like proteins in oats cause inflammation weakly and in only a few individuals who are predisposed to develop celiac disease. Rice and corn do not cause celiac disease because they do not absorb gliadin-like proteins.
What does celiac disease do to the small intestine?

The small intestine has an inner lining of cells that form finger-like projections called villi. The villi are important because they increase the number of cells and surface dwelling available for the absorption of nutrients from the intestinal lumen into the blood stream. In celiac disease, the inflammation destroys the villi, causing the inner lining of the petite intestine to become flattened. This loss of villi reduces the cells and surface area available for absorption of nutrients. The impaired absorption of nutrients is referred to as malabsorption. The malabsorption of nutrients leads to nutrient deficiencies, referred to as malnutrition.

The length (amount) of the small intestine affected by the loss of villi varies from patient to patient, and the length that is involved determines the severity of signs and symptoms. Thus, patients whose entire cramped intestine is affected by a loss of villi have more severe signs and symptoms of malabsorption than patients who have only part of the small intestine affected. When only part of the small intestine is affected, it usually is the upper small intestine (the duodenum and jejunum) that is more affected than the lower minute intestine (the ileum).

What are the signs and symptoms of celiac disease?

Depending on the degree of malabsorption, the signs and symptoms of celiac disease vary among individuals, ranging from no symptoms, few or peaceful signs and symptoms, to many or severe signs and symptoms. There are two categories of signs and symptoms: 1) signs and symptoms due to malabsorption, and 2) signs and symptoms due to malnutrition including vitamin and mineral deficiencies.

1. Signs and symptoms of malabsorption

The three major categories of dietary nutrients are carbohydrates, proteins, and burly. Absorption of all of these nutrients can be reduced in celiac disease; however, fat is the most commonly and severely affected nutrient. Most of the gastrointestinal symptoms and signs of celiac disease are due to the inadequate absorption of fat (fleshy malabsorption). Gastrointestinal symptoms of fat malabsorption include diarrhea, malodorous flatulence (foul smelling gas), abdominal bloating, and increased amounts of fat in the stool (steatorrhea). The unabsorbed fat is broken down by intestinal bacteria into fatty acids, and these fatty acids promote secretion of water into the intestine, resulting in diarrhea. Fatty stools typically are large in volume, malodorous (foul smelling), greasy, light tan or light grey in color, and tend to float in the toilet bowl. Oil droplets (undigested fat) also may be seen floating on top of the water.

Loss of intestinal villi also causes malabsorption of carbohydrates, particularly the sugar lactose. Lactose is the primary sugar in milk. Lactose is made up of two smaller sugars, glucose and galactose. In order for lactose to be absorbed from the intestine and into the body, it must first be split into glucose and galactose. The glucose and galactose then can be absorbed by the cells lining the small intestine. The enzyme that splits lactose into glucose and galactose is called lactase, and it is located on the surface of the small intestinal villi. In celiac disease the intestinal villi along with the lactase enzymes on their surface are destroyed, leading to malabsorption of lactose.

Signs and symptoms of malabsorption of lactose are particularly prominent in individuals with celiac disease who have underlying lactose intolerance, a genetically determined reduction in the activity of lactase. Symptoms of lactose malabsorption (diarrhea, excessive flatulence (passing gas), abdominal pain and abdominal bloating or distension) occur because unabsorbed lactose passes through the small intestine and into the colon. In the colon, there is a normal bacterium that contains lactase and is able to split the lactose, using the resulting glucose and galactose for its own purposes. Unfortunately, when they split the lactose into glucose and galactose, the bacteria also release gas (hydrogen and /or methane). A proportion of the gas is expelled and is responsible for the increased flatus (passing gas) that may occur in celiac disease. Increased gas mixed in the stool is responsible for stool floating in the toilet bowl.

Not all of the lactose that reaches the colon is split and used by colonic bacteria. The unsplit lactose that reaches the colon causes water to be drawn into the colon (by osmosis). This promotes diarrhea.

2. Signs and symptoms of malnutrition and vitamin or mineral deficiencies

Symptoms of malnutrition and vitamin or mineral deficiencies include: weight loss, fluid retention, anemia, osteoporosis, bruising easily, peripheral neuropathy (nerve damage), infertility, and muscle weakness.

• Weight loss and fluid retention: Weight loss is the direct result of inadequate absorption of carbohydrates, proteins and fat. However, weight loss may not always occur because patients with celiac disease often have enormous appetites that compensate for the reduced absorption of nutrients. Moreover, weight loss can be masked by fluid retention. Fluid retention occurs in advanced malnutrition because the reduced absorption of protein results in low protein levels in the blood. High protein levels in the blood are necessary to keep fluid from leaking out of blood vessels and into the body’s tissues. When blood protein levels fall as in celiac disease, fluid leaks into many tissues (edema) but particularly the ankles and feet, which swell due to the edema.

• Anemia: Lack of absorption of vitamin B12 and iron can lead to anemia.

• Osteoporosis: Lack of absorption of vitamin D and calcium can lead to osteoporosis and bone fractures.

• Easy bruising: Lack of absorption of vitamin K can lead to diminished ability of blood to clot and hence to easy bruising or excessive bleeding.

• Peripheral neuropathy (nerve damage): Vitamin deficiencies of B12 and thiamine may contribute to nerve damage with symptoms of poor balance, muscle weakness, and numbness and tingling in the arms and legs.

• Infertility: Untreated celiac disease can lead to infertility in women, lack of menses (menstruation), spontaneous abortions and low birth weight infants.

• Muscle weakness: Lack of absorption and low levels of potassium and magnesium can lead to severe muscle weakness, muscle cramps, and numbness or tingling sensations in the arms and legs.

How do symptoms of celiac disease differ with age of onset?

In the past, celiac disease was considered to be a disease primarily of infants and children. It is now clear that initial signs and symptoms of celiac disease can occur in adults and even in the elderly.

Symptoms in infants

Infants with celiac disease typically have diarrhea, steatorrhea, abdominal cramps, abdominal distension, irritability, muscle wasting, and failure to thrive and grow. These symptoms typically occur after introduction of gluten-containing cereals into their diets.

Symptoms in children

Children with celiac disease typically have diarrhea, increased amounts of fat in the stool (steatorrhea), flatulence (passing gas), short stature and weight loss. Trustworthy treatment with a gluten-free diet can lead to accelerated (catch-up) growth in height; however, if untreated, childhood celiac disease can result in short stature as an adult. As children with celiac disease enter adolescence, many will experience spontaneous remissions (reduced symptoms) and remain free of the signs and symptoms of celiac disease until later in adulthood. This later reactivation can be precipitated by stress such as pregnancy or surgery.

Symptoms in adults

Adults with celiac disease may have symptoms of diarrhea, steatorrhea, weight loss and flatulence; however, many adults do not have diarrhea or steatorrhea. They have either no symptoms or only vague abdominal discomfort such as bloating, abdominal distension and excess gas. They also may have only one, or only a few signs of malnutrition such as iron deficiency anemia, abnormal bleeding, or bone fractures. Some patients with celiac disease and gastrointestinal symptoms are mistakenly diagnosed with short-tempered bowel syndrome (IBS).

There have been changes during the past 20 years in the way in which celiac disease is diagnosed. The average age at which celiac disease is diagnosed has increased, probably because of the increased awareness that the disease can first cause symptoms or signs in adults. Whereas diarrhea was the initial symptom in 80% of patients, it now is the initial symptom in only 40%. A small proportion of patients-about 15%–are now diagnosed with blood antibody tests because they have a close relative with celiac disease and they are being screened to perceive if they also have the disease.
What is latent and silent celiac disease?

The terms latent and silent celiac disease are used to refer to patients who have inherited the genes that predispose them to celiac disease but have not yet developed the symptoms or signs of celiac disease.

Latent celiac disease refers specifically to patients who have abnormal antibody blood tests for celiac disease (see discussion of specific tests for celiac disease) but who have normal small intestines and no signs or symptoms of celiac disease. For example:

• Some patients may have had a childhood onset of celiac disease and the disease may have been successfully treated with a gluten-free diet. The patients’ intestines may have resumed a normal appearance and function, and they may have no signs or symptoms of celiac disease.

• Some patients with celiac disease in childhood abandon the gluten free diet as adults, yet they remain free of the signs or symptoms of celiac disease.

In both of the above instances, the celiac disease is latent, and the patients can effect signs and symptoms of celiac disease later in life.

Silent celiac disease refers to patients who have abnormal antibody blood tests for celiac disease as well as loss of villi in the small intestine but have no symptoms or signs of celiac disease, even on a normal diet that contains gluten. Like patients with latent celiac disease, these patients can develop signs or symptoms of celiac disease later in life.

What diseases are associated with celiac disease?

The following are diseases associated with celiac disease:

• An estimated 10% of patients with celiac disease also have dermatitis herpetiformis. Dermatitis herpetiformis is a disease of the skin that is characterized by an itchy rash on the extremities, buttocks, neck, trunk, and scalp.

• Recurrent painful mouth ulcers (aphthous stomatitis)

• Insulin-dependent diabetes (juvenile-onset or type 1 diabetes)

• Autoimmune thyroid disease

• Rheumatoid arthritis

• Systemic lupus

How is celiac disease diagnosed?

Celiac disease is suspected when individuals have signs or symptoms of malabsorption or malnutrition. Other diseases, however, can produce malabsorption and malnutrition, for example, pancreatic insufficiency (a pancreas that is not able to produce digestive enzymes), Crohn’s disease of the dinky intestine, and small intestinal overgrowth of bacteria. It is important, therefore, to confirm suspected celiac disease with appropriate testing.

Small intestinal biopsy

Small intestinal biopsy is considered the most accurate test for celiac disease. Runt intestinal biopsies can be obtained by performing an esophagogastroduodenoscopy (EGD). During an EGD, the doctor inserts a long, flexible viewing endoscope through the mouth and into the duodenum. A long, flexible biopsy instrument then can be passed through a small channel in the endoscope to obtain samples of the intestinal lining of the duodenum. Multiple samples usually are obtained to increase the accuracy of diagnosis. A pathologist then can examine the biopsies (under a microscope) for loss of villi and other characteristics of celiac disease such as increased numbers of lymphocytes.

Small intestinal biopsy does however, have some limitations. For example, acute viral gastroenteritis and allergy to cow’s milk or soy protein can cause abnormal small intestinal biopsies that are indistinguishable from celiac disease. However, acute viral gastroenteritis is not easily confused with celiac disease because of the contrast in the acuteness of symptoms. (Acute viral gastroenteritis has a sudden onset of symptoms and last only a few days.) It is however, easier to confuse cow’s milk and soy protein allergies with celiac disease, but these allergic conditions are rare and primarily occur in young children. Despite these limitations, small intestinal biopsies are recommended even for individuals who have abnormal antibody tests for celiac disease. (See discussion that follows.)

Specific antibody tests for celiac disease

Antibodies are proteins that are produced by the immune system to fight viruses, bacteria, and other organisms that infect the body. Sometimes, however, the body produces antibodies against non-infectious substances in the environment (for example, in hay fever) and even against its own tissues (autoimmunity).

Blood tests that are specific for celiac disease include endomysial antibodies, anti-tissue transglutaminase antibodies, and anti-gliadin antibodies. In patients with celiac disease, anti-gliadin antibody is an antibody produced against gliadin in the diet and endomysial and anti-tissue transglutaminase antibodies are antibodies produced against the body’s own tissues.

Endomysial antibodies and anti-tissue transglutaminase antibodies are highly gracious in diagnosing celiac disease. An individual with abnormally elevated endomysial and anti-tissue transglutaminase antibodies has a greater than 95% chance of having celiac disease. Anti-gliadin antibodies are less reliable and have a high false positive rate. Thus a person with an abnormally elevated anti-gliadin antibody level does not necessarily have celiac disease. Nevertheless, anti-gliadin antibody levels are useful in monitoring the response to treatment because anti-gliadin antibody levels usually begin to fall within several months of successful treatment of celiac disease with a gluten free diet.
Who should undergo antibody blood tests for celiac disease?

Some experts recommend that antibody blood tests be used to screen healthy persons with no signs or symptoms for celiac disease. In Italy, where celiac disease is approved, all children are screened for celiac disease. Experts in the United States do not recommend screening healthy persons for celiac disease. Antibody blood tests are only recommended for individuals with a higher likelihood than normal of having celiac disease. These patients are:

1. Patients with chronic diarrhea (diarrhea that does not resolve in three weeks), increased amount of plump in the stool (steatorrhea), and weight loss

2. Patients with excess gas, bloating, and abdominal distension

3. First and second degree relatives of patients who have celiac disease

4. Children with growth retardation

5. Patients with unexplained iron deficiency anemia

6. Patients with skin rashes suggestive of dermatitis herpetiformis

7. Patients with recurrent painful mouth sores (aphthous stomatitis)

8. Patients with disease known to be associated with celiac disease. Examples of these diseases include insulin-dependent diabetes mellitus, autoimmune thyroid disease, rheumatoid arthritis, systemic lupus, ulcerative colitis, etc.

Why is it important to accurately diagnose celiac disease?

Diagnosis of celiac disease should be firmly established before commencing treatment with a gluten free diet for several reasons.

1. The gluten free diet is a life-long and tedious commitment that should not be taken lightly. It is more costly than a normal diet and has significant social implications for dining out.

2. Patients with irritable bowel syndrome (IBS) may experience improvements in bloating, abdominal pain, and diarrhea with a gluten free diet. These patients may be misdiagnosed as having celiac disease. Without confirmation of celiac disease by small intestinal biopsy, they may be unnecessarily committed to life-long gluten restriction.

3. A gluten free diet can lower blood antibody levels and allow the microscopic appearance of the small intestine to lose the typical appearance of celiac disease, complicating subsequent efforts at making a firm diagnosis of celiac disease.

How are malabsorption and malnutrition evaluated in celiac disease?

Celiac disease causes malabsorption of nutrients and leads to malnutrition. Tests are available that serve in the evaluation of malabsorption and malnutrition; however, because other diseases can cause both malabsorption and malnutrition, these tests cannot be venerable to diagnose celiac disease.

Stool examination for malabsorption

Elephantine in a sample of stool placed on a glass slide can be stained with a dye (Sudan stain) to make the fat visible under the microscope as globules. Stool from patients with celiac disease often contains many stained globules of fat, and Sudan staining is a quick and easy screening test for increased amounts of fat in the stool (steatorrhea). To conclusively diagnose steatorrhea, however, stool is unruffled over a 72-hour period, and the fat in the stool is chemically measured and quantified. Steatorrheic stools have abnormally high quantities of fat. Since malabsorption and steatorrhea can occur with other intestinal diseases (such as limited intestinal bacteria overgrowth, prior minute intestinal resection, extensive Crohn’s disease of the small intestine, and chronic pancreatitis), stools with substantial amounts of fat only raise the suspicion of celiac disease but cannot be used to diagnose celiac disease.

Blood tests for malnutrition and vitamin deficiencies

Malabsorption reduces the absorption of protein and causes a reduction in blood protein levels. This can be seen commonly as a reduced blood level of albumen, the most concentrated protein in blood. Other proteins in blood, for example, pre-albumen and transferrin also may be reduced.

Intestinal malabsorption can lead to deficiencies and low blood levels of iron, calcium, vitamin B12, folate, Vitamin D and vitamin K. These deficiencies, in turn, can lead to other blood test abnormalities such as:

1. Iron deficiency anemia: Iron is an important component of hemoglobin in red blood cells. When iron is deficient, production of red blood cells is impaired, and anemia develops. Iron deficiency anemia can occur either through loss of blood (with its iron-containing red blood cells) or lack of intestinal iron absorption. Heavy menstrual bleeding and cancer of the colon that bleeds into the intestine are two common causes of iron deficiency anemia due to blood loss. Celiac disease causes iron deficiency anemia by reducing intestinal iron absorption. In fact, iron deficiency anemia can be an important clue to the presence of celiac disease.

2. Abnormally prolonged prothrombin time (ProTime): ProTime is a blood test that measures how quickly blood clots. Clotting of blood requires special proteins or clotting factors, many of which are made by the liver. Formation of clotting factors by the liver requires vitamin K. When vitamin K absorption from the intestine is reduced, as in celiac disease, the production of clotting factors by the liver is inadequate, and blood clotting is delayed. Delayed clotting is reflected in an abnormal ProTime, and individuals with an abnormal ProTime have a higher risk of abnormal or excessive bleeding.

Iron deficiency anemia, abnormal ProTime, steatorrhea, and low iron and vitamin levels can occur in diseases other than celiac disease. Therefore the presence of these abnormalities only raises the suspicion of celiac disease but does not specifically diagnose celiac disease.

What is the treatment of celiac disease?

There is no cure for celiac disease. The treatment of celiac disease is a gluten free diet. Celiac disease patients vary in their tolerance of gluten; some patients can ingest small amounts of gluten without developing symptoms while others experience massive diarrhea with only miniature amounts of gluten. The standard treatment of disease patients calls for complete avoidance of gluten for life. The principles of a gluten free diet include:

1. Avoid all foods made from wheat, rye, and barley. Examples are breads, cereals, pasta, crackers, cakes, pies, cookies, and gravies.

2. Avoid oats. Some patients with celiac disease can tolerate oats in the diet. But long-term safety of oats in celiac disease patients is unknown. Also some oat preparations can be unpleasant with wheat. Thus, it is probably best to avoid oats at least during the initial treatment with a gluten free diet. Once disease remission is achieved with a strict gluten free diet, small quantities of oats can be reintroduced into the diet under medical supervision.

3. Pay attention to processed foods that may contain gluten. Wheat flour is a common ingredient in many processed foods. Examples of foods that may contain gluten, to name only a few, include:
   

   • canned soups,
   • salad dressings,
   • ice cream,
   • candy bars,
   • instant coffee,
   • luncheon meats,
   • ketchup,
   • mustard,
   • processed and canned meats,
   • yogurt,
   • sausages and,
   • pasta.

4. Beware of tablets, capsules, and vitamin preparations that bear gluten. Wheat starch is commonly employed as a binding agent in tablets and capsules. Gluten also can be found in many vitamin products, and cosmetic products such as lipstick.

5. Avoid beer

6. It is all right to drink wine, brandy, whiskey and other non-wheat or barley alcohol (in moderation!)

7. Avoid milk and other dairy products that possess lactose. Untreated patients with celiac disease often are lactose intolerant. With successful treatment, dairy products can be reintroduced slowly into the diet later.

8. It is alright to consume fish, fresh meats, rice, corn, soybean, potato, poultry, fruits, vegetables, and dairy products (for patients who are not lactose intolerant)

9. Consult dietitians and national celiac disease societies for lists of gluten free foods. Read the food and product labels before buying or consuming any product. This is necessary because a manufacturer may change a product’s ingredients at any time. A product that was gluten-free in the past may now contain gluten. Even branded products may be gluten free in one country but contain gluten in another country. If one is not certain after reading the labels, call the manufacturer.

10. Because patients with severe malabsorption can develop vitamin and mineral deficiencies, vitamin and mineral supplements are famous. All patients should take a multivitamin daily. Patients with iron deficiency anemia should be treated with iron. Patients with anemia due to folate or B12 deficiency should be treated with folic acid and B12. Patients with an abnormal ProTime should be treated with vitamin K. Patients with uncouth blood calcium levels or with osteoporosis should be treated with calcium and vitamin D supplements.

In most patients, a gluten free diet will result in improvements in symptoms within weeks. Many patients report symptom improvements within 48 hours. In children with celiac disease, the response to a gluten free diet can be dramatic. Not only will diarrhea and abdominal discomfort subside, but behavior also improves, and growth resumes (with rapid catch up in height). These improvements in symptoms are followed by reappearance of intestinal villi. Complete normalization of the intestinal villi may take months. In many adult patients, the improvement in symptoms is followed by only partial regeneration of intestinal villi. In patients with dermatitis herpetiformis, the skin lesions also improve with a gluten free diet.

Many patients with celiac disease may not understand the importance of life-long adherence to a gluten free diet. A recent study found that among patients diagnosed at least 20 years earlier with celiac disease, only half of the patients were following a strict gluten-free diet. The primary reason that patients followed the diet was to prevent symptoms-not to prevent complications. There was evidence of mild iron deficiency and abnormal bone density each in one-third of the patients, suggesting that the lack of adherence to the diet was having health consequences.

What if patients fail to respond to gluten free diet?

Failure to respond to a gluten free diet can be due to several reasons:

1. Patients are not following a strict gluten free diet and are detached eating small amounts of gluten.

2. Patients are unknowingly ingesting unsuspected sources of gluten such as starch, binders and fillers in medications or vitamins.

3. Patients may have another co-existing condition such as moody bowel syndrome, bacterial overgrowth of the small bowel, microscopic colitis, or pancreatic insufficiency that are causing the symptoms.

4. Patients may have refractory disease, or complications of celiac disease.

What is refractory celiac disease?

Refractory celiac disease is a rare condition in which the symptoms of celiac disease (and the loss of villi) do not improve despite many months of a strict gluten free diet. Before making a diagnosis of refractory celiac disease it is important to exclude complications of celiac disease and other co-existing conditions that can manufacture similar symptoms. It is believed by many knowledgeable physicians that refractory celiac disease is a malignant condition, that is, it is a cancer.

What is the treatment for refractory celiac disease?

The treatment of refractory celiac disease is first to make obvious that all gluten is eliminated from the diet. If there still is no improvement, medications are used.

• Corticosteroids such as prednisone have been used successfully in treating some patients with refractory celiac disease.

• Immuno-suppressive drugs (medications that suppress a person’s immune system) such as azathioprine (Imuran, Azasan) and cyclosporine also have been used. (These drugs also are used in treating some types of cancer.)

• Corticosteroids and immunosuppressive drugs are potent medications with potentially serious side effects. Many patients with refractory celiac disease are malnourished and have weakened immune systems, and corticosteroids and immunosuppressive agents can further increase their risk of serious infections. Thus doctors experienced with treating celiac disease should monitor treatment of refractory celiac disease.

Unfortunately in some patients with refractory celiac disease, malabsorption and malnutrition progress despite drugs. In these patients the intravenous route is the only way to deliver nutrition. Total parenteral nutrition (TPN) is a way of delivering calories, carbohydrates, amino acids, and fat in liquid solutions via a catheter that has been inserted and secured into a vein.
What are the complications of celiac disease?

The complications of celiac disease include cancers, small bowel ulcers (ulcerative jejunoileitis), and collagenous celiac disease.

Cancer

Adults with celiac disease have a several-fold higher than normal risk of developing lymphomas (cancers of the lymph glands) in the small intestine and elsewhere. They also have a high risk of small intestinal and, to a lesser degree, of esophageal carcinomas (cancers of the inner lining of the intestine and esophagus). Lymphoma tends to develop in adult patients who have had celiac disease for longer than 20-30 years and in patients with refractory celiac disease. Symptoms of slight intestinal lymphoma or carcinoma include anemia, bleeding into the intestine, abdominal pain, weight loss, fever, and little intestinal obstruction (with symptoms of abdominal distension, vomiting and crampy abdominal pain). Small intestinal lymphoma and carcinoma are difficult to diagnose. Sometimes in patients with celiac disease, where the disease has been controlled with a gluten free diet, recurrence of weight loss, anemia, abnormal pain, and symptoms of intestinal obstruction will lead doctors to search for intestinal lymphoma and carcinoma.

Computerized tomography (CT) scans of the abdomen, enteroclysis (one type of barium x-ray of the cramped intestine), and enteroscopy (inspection of the small intestine using a long, flexible endoscope) are procedures doctors use to diagnose small intestinal lymphoma and carcinoma. Sometimes diagnoses of intestinal lymphoma or carcinoma can only be made with surgery (open laparotomy) or by laparoscopy (examination of the abdominal cavity with flexible endoscopes). The prognosis for patients who compose intestinal lymphoma usually is dreadful. Long-term survival (survival beyond 5 years) of patients with small intestinal lymphoma is estimated to be only 10%. Other cancers that may be increased in patients with celiac disease include cancers of the liver, oral cavity, and large intestine.

Ulcerative jejunoileitis

Ulcerative jejunoileitis is a rare complication of celiac disease. In ulcerative jejunoileitis there are recurrent episodes of small intestinal ulcerations and formation of strictures (narrowing of the intestinal lumen). Small intestinal ulcerations and stricture formation can lead to intestinal bleeding, weight loss, abdominal pain, and intestinal obstruction. Patients with ulcerative jejunoileitis are at high risk of developing intestinal lymphomas. The diagnosis of ulcerative jejunoileitis is made by enteroclysis of the itsy-bitsy intestine, enteroscopy, or CT scan of the abdomen. Treatment involves a gluten free diet and surgical resection of the most diseased portions of the itsy-bitsy intestine. The prognosis is poor; long-term survival for patients with ulcerative jejunoileitis beyond 5 years is less than 50%.

Collagenous celiac disease

Collagenous celiac disease is a rare, but serious complication of celiac disease in which a patient may have the symptoms of celiac disease initially, but they fail to improve on a gluten free diet, and after several years a large amount of scar tissue (collagen) forms just under the intestinal lining. There is no treatment for collagenous celiac disease, and the prognosis is poor.
Can cancer risk be reduced in celiac disease?

Some doctors believe that strict adherence to a gluten free diet can crop the risk of cancer in patients with celiac disease, but further studies are needed to present this. Until more is known in this area, patients with celiac disease should adhere strictly to a gluten free diet.

What’s new in celiac disease?

The diagram in which gluten and gliadin cause disease appears to be complex. It does not appear to be simply a matter of an immune response to gliadin. Current information suggests that gliadin in the diet is altered by tissue transglutaminase in the little intestine. This altered gliadin is what provokes the immunologic response that leads to the production of antibodies to tissue transglutaminase and the inflammation that destroys the villi.

Symptoms of celiac disease can be similar to those of short-tempered bowel syndrome (IBS), and the issue often arises if patients with IBS need to be screened for celiac disease. If they are screened, should they be screened with blood antibody tests, microscopic intestinal biopsies, or both? One study in particular has addressed this issue. Approximately 100 patients thought to have diarrhea from IBS were studied. Among the IBS patients, none had celiac disease-associated antibodies in their blood, but 30% had the antibodies in juice obtained from within the duodenum. Twenty-three percent of the patients with IBS had lymphocytes in the lining of the small intestine just like patients with celiac disease. Finally, 35% of the IBS patients had the genes that commonly are found in celiac disease. These engrossing findings need to be confirmed by additional studies. If confirmed, they would suggest that a proportion of patients with IBS may actually have celiac disease, and that the diagnosis might require small intestinal biopsy and antibody studies of duodenal juice.

Celiac Disease At a Glance

• Celiac disease is a chronic digestive disorder in which damage to the lining of the small intestine leads to the malabsorption of minerals and nutrients.

• The destruction of the inner lining of the small intestine in celiac disease is caused by an immunological (allergic) reaction to gluten.

• Gluten is a family of proteins present in wheat, barley, rye, and sometimes oats.

• Patients with celiac disease may develop diarrhea, steatorrhea, weight loss, flatulence, iron deficiency anemia, abnormal bleeding, or weakened bones. However, many adults with celiac disease may have either no symptoms or only vague abdominal discomfort such as bloating, abdominal distension and excess gas.

• Children with celiac disease may also have stunted growth, and if untreated, childhood celiac disease can result in short stature as an adult.

• Small intestinal biopsy is considered the most accurate test for celiac disease.

• Blood tests can be performed to diagnose celiac disease; they include endomysial antibodies, anti-tissue transglutaminase antibodies, and anti-gliadin antibodies.

• There is no cure for celiac disease. The treatment of celiac disease is a gluten free diet.

• In most patients, a gluten free diet will result in improvements in symptoms within weeks. Many patients report symptom improvements within 48 hours.

• In children with celiac disease, successful treatment with gluten free diet can also lead to resumption of growth (with rapid catch up in height).

• Failure to respond to gluten free diet can be due to several reasons: the most common reason is failure to adhere to a strict gluten free diet.

• Refractory celiac disease is a rare condition in which the symptoms of celiac disease (and the loss of villi) do not improve despite many months of a strict gluten free diet.

• The treatment of refractory celiac disease is first to make sure that all gluten is eliminated from the diet. If there serene is no improvement, corticosteroids such as prednisone, and immunosuppressive agents (medications that suppress a person’s immune system) such as azathioprine and cyclosporine have been used.

• Adults with celiac disease have a several-fold higher than normal risk of developing lymphomas (cancers of the lymph glands) in the small intestine and elsewhere. They also have a high risk of small intestinal and, to a lesser degree, of esophageal carcinomas (cancers of the inner lining of the intestine and esophagus).

• The prognosis for celiac disease patients who gain lymphoma, collagenous celiac disease, or jejunal ulcers is poor.

Tags: hodgkins lymphoma awareness, Lymphoma Awareness, non hodgkins lymphoma awareness

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