Celiac disease (CD) is an enteropathy-induced immune response that occurs on exposure to toxic gluten in the diet and is reversible once gluten is withdrawn. A gluten-free diet is the preferred treatment for CD and leads to reversal of villous atrophy. Counseling, nutritional support, and follow-up are vital aspects in CD management. The pickup rate of CD has improved with the availability of serologic tests, and this has led to a reduction in morbidity in treated CD cases. Managing CD can potentially prevent or cure some of the associated conditions, such as neurologic complications, nutritional deficiencies, and osteoporosis.
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Celiac disease (CD) is an enteropathy-induced immune response that occurs on exposure to toxic gluten in the diet and is reversible once gluten is withdrawn.
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A gluten-free diet is the preferred treatment for CD and leads to reversal of villous atrophy.
- •
Counseling, nutritional support, and follow-up are vital aspects in CD management.
- •
The pickup rate of CD has improved with the availability of serologic tests, and this has led to a reduction in morbidity in treated CD cases.
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Managing CD can potentially prevent or cure some of the associated conditions, such as neurologic complications, nutritional deficiencies, and osteoporosis.
Introduction
Celiac disease (CD) is an abnormal immune response to ingestion of gluten, which results in small intestine inflammation. The clinical and histologic manifestations of this process are potentially reversible when gluten is excluded from the diet. Gluten is a generic term used to collectively describe all the cereal proteins that are toxic to individuals with CD. Wheat, barley, and rye have all been shown to cause intestinal inflammation in patients with CD. The classical presentation historically referred to a disease occurring in children younger than 2 years characterized by symptoms of malabsorption and poor growth. These events typically occur after gluten is introduced into the diet. A trend is being seen toward fewer patients presenting with symptomatic CD characterized by diarrhea, with a significant shift toward more patients presenting as asymptomatic adults with CD detected at screening.
CD can be associated with other conditions, such as autoimmune disorders and malignancy. The morbidity and mortality of patients with untreated CD is higher compared with those of healthy individuals. Rubio-Tapia and colleagues studied 9133 healthy young adults at Warren Air Force Base (sera were collected between 1948 and 1954) and 12,768 gender-matched subjects from 2 recent cohorts from Olmsted County, Minnesota, with either similar years of birth (n = 5558) or age at sampling (n = 7210) to that of the Air Force cohort. Sera were tested for tissue transglutaminase and, if abnormal, for endomysial antibodies. Survival was measured during a follow-up period of 45 years in the Air Force cohort. The prevalence of undiagnosed CD was 0.2%. During 45 years of follow-up in the older cohort, all-cause mortality was nearly 4-fold greater in persons with undiagnosed CD than among those who were seronegative.
The standard treatment for CD is adhering to a gluten-free diet (GFD), which involves avoiding substances containing toxic gluten, such as wheat, rye, and barley products. Evidence has shown the long-term safety of oats as part of a GFD in patients with CD. However, 5% to 10% may experience a response, because some people have small intestinal T cells that react to oat avenins.
It is equally important to educate patients about the disease diagnosis and prognosis, which increases their knowledge about their condition and improves their adherence to the GFD. Patients should be counseled by a dietitian knowledgeable about the condition. Morbidities associated with CD must be addressed and managed when necessary, and preventative measures should also be taken, as detailed in the following sections.
Dietary treatment
Gluten Exclusion
The cornerstone of therapy is lifelong adherence to a GFD, which involves the exclusion of foods containing wheat, rye, and barley. Gluten-free foods by definition should not contain more than 20 mg/kg of gluten. In one study, as little as 50 mg/d was sufficient to cause mucosal damage after 3 months. Ingestion of oats is generally safe in CD, but because oats are often contaminated with toxic gluten from wheat and other grains, only oats from a dedicated gluten free source are recommended for inclusion in the GFD. Evidence suggests that oats are not harmful to most individuals with CD. However, most commercially available oat flour is contaminated with 10% to 15% wheat.
Avoiding cereals containing toxic gluten is a formidable task, because these are found in bread, biscuits, cakes, pastries, breakfast cereals, pasta, beer, and most soups, sauces, and puddings.
Gluten elimination leads to some lifestyle restrictions. As a result, compliance with a strict GFD is limited. Noncompliance is especially likely in patients who are less symptomatic or have better tolerance to gluten. The most common reasons for a lack of response are poor compliance or inadvertent gluten ingestion ( Box 1 ).
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Sausages and beef burgers
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Luncheon meat
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Imitation crab meat
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Gravy powder and browning
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Self-basting turkeys
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Soups
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Brown rice syrup
- •
Soy sauce and other sauces
- •
Chutneys and pickles
- •
White pepper
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Supplements
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Some pharmaceutical products
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Oatmeal contaminated with gluten
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Communion wafers
- •
Instant coffee
- •
Potato crisps/chips
- •
Licorice
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Curry powder
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Some medicines containing starch or wheat derivatives
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Farina
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Matzo flour/meal
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Hydrolyzed vegetable protein
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Meat and fish pastes
- •
Salad dressings
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Hard candy
- •
Some toothpastes
- •
Play dough
- •
Some lipsticks
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Malt vinegar
- •
Some chocolates and drinking chocolate
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Mustards
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Blue cheese
- •
Shredded suet
- •
Baked beans
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Paté
- •
Seitan
All patients should see a dietician with experience in CD. The dietician should provide formal education about following a GFD. Attention should be given to food labeling and potential pitfalls, and counseling should be provided on weight management. Written information ideally should be provided, and referral to a support group is helpful because it provides individual with CD access to emotional and psychological support and information on the latest GFD products. Through these support groups, patients can share their experiences and develop the sense of not being alone in dealing with this disease. Ideally, a second appointment should be offered after 3 to 6 months to check and answer any questions that have arisen and assess adherence. Further details regarding dietary assessment are discussed in a separate article elsewhere in this issue (“Dietary Assessment in Celiac Disease”).
Patients may supplement their diet with commercial gluten-free products. Many gluten-free flours and bread mixes are based on purified wheat starch from which most gluten proteins have been removed. Most patients with CD tolerate these products well, although a minority cannot. These individuals should be advised to follow a strict GFD for which they ingest only commercial non–wheat starch–based gluten-free products. Symptomatic patients may be advised to avoid excessive milk ingestion in the first 6 months of following a GFD. Up to 70% of individuals with CD respond promptly to a GFD, showing improvement of symptoms within weeks or days. In one study of 158 patients with CD evaluating the histologic recovery after initiation of a GFD showed an improvement in 65% of the patients within 2 years, 85.3% within 5 years, and 89.9% after 5 years of follow-up.
Adhering to a strict GFD is particularly important, not only to improve symptoms and general well-being but also because of the significant effects on morbidity and mortality. With continued gluten ingestion, the resulting enteropathy may eventually lead to complications, such as osteoporosis, anemia, and vitamin D, copper, and zinc deficiency. These deficiencies can be partially reversed with a GFD. Prompt and strict dietary treatment decreases all-cause mortality in patients with CD who adhere to a GFD.
Some gluten-free products may contain trace amounts of gluten, and gluten contamination can also occur. This ingestion does not necessarily lead to treatment failure. The accepted daily intake of gluten in CD is 20 parts per million (ppm). One study involving 76 adults with CD evaluated the intake of 59 naturally gluten-free and 24 wheat starch–based gluten-free products. The gluten in these diets was quantified, and the intake of these products was compared with mucosal histology. Several naturally gluten-free (13 of 59) and wheat starch–based gluten-free (11 of 24) products were found to contain gluten from 20 to 200 ppm. The median daily flour consumption was 80 g (range: 10–300 g). Within these limits, the long-term mucosal recovery was good. Results of this study suggested that the safe level of gluten consumption is 100 ppm. The exact daily gluten intake is difficult to quantify, because the actual gluten content of a typical GFD is unknown. Some medications may contain minimal gluten and must be avoided.
Total Versus Partial Gluten Exclusion
The risk of developing small intestinal lymphoma is increased in patients with CD who ingest a diet containing gluten. Nutritional deficiencies are also more likely to occur. Therefore, recommending a strict GFD seems reasonable, even if patients are asymptomatic with a low gluten intake. Good dietary compliance should reduce the risk of osteoporosis in later life.
Need for Lifelong Treatment
Some adolescents might stop adhering to a GFD because they mistaken believe that they have “grown out of” their CD. If the diagnosis is doubted, a gluten challenge and repeat jejunal biopsy should be undertaken. If the diagnosis is established, lifelong treatment should be recommended.
Information and Support
Adherence to a GFD is not easy. Adolescence is often a particularly difficult time, when young patients may feel excluded from their peer group by their dietary restriction. Therefore, careful explanation and counseling is of great value, and the role of dietitians is vital to this process. Written advice should be provided whenever possible, and all patients should be encouraged to join celiac support groups.
Dietary treatment
Gluten Exclusion
The cornerstone of therapy is lifelong adherence to a GFD, which involves the exclusion of foods containing wheat, rye, and barley. Gluten-free foods by definition should not contain more than 20 mg/kg of gluten. In one study, as little as 50 mg/d was sufficient to cause mucosal damage after 3 months. Ingestion of oats is generally safe in CD, but because oats are often contaminated with toxic gluten from wheat and other grains, only oats from a dedicated gluten free source are recommended for inclusion in the GFD. Evidence suggests that oats are not harmful to most individuals with CD. However, most commercially available oat flour is contaminated with 10% to 15% wheat.
Avoiding cereals containing toxic gluten is a formidable task, because these are found in bread, biscuits, cakes, pastries, breakfast cereals, pasta, beer, and most soups, sauces, and puddings.
Gluten elimination leads to some lifestyle restrictions. As a result, compliance with a strict GFD is limited. Noncompliance is especially likely in patients who are less symptomatic or have better tolerance to gluten. The most common reasons for a lack of response are poor compliance or inadvertent gluten ingestion ( Box 1 ).
- •
Sausages and beef burgers
- •
Luncheon meat
- •
Imitation crab meat
- •
Gravy powder and browning
- •
Self-basting turkeys
- •
Soups
- •
Brown rice syrup
- •
Soy sauce and other sauces
- •
Chutneys and pickles
- •
White pepper
- •
Supplements
- •
Some pharmaceutical products
- •
Oatmeal contaminated with gluten
- •
Communion wafers
- •
Instant coffee
- •
Potato crisps/chips
- •
Licorice
- •
Curry powder
- •
Some medicines containing starch or wheat derivatives
- •
Farina
- •
Matzo flour/meal
- •
Hydrolyzed vegetable protein
- •
Meat and fish pastes
- •
Salad dressings
- •
Hard candy
- •
Some toothpastes
- •
Play dough
- •
Some lipsticks
- •
Malt vinegar
- •
Some chocolates and drinking chocolate
- •
Mustards
- •
Blue cheese
- •
Shredded suet
- •
Baked beans
- •
Paté
- •
Seitan
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