Therapy and Management of Celiac Disease

Medical Care

Total lifelong avoidance of gluten ingestion is the cornerstone treatment for patients with celiac disease (CD). Wheat, rye, and barley are the grains that contain toxic peptides. They should be eliminated as completely as possible, although daily intake doses larger than 10 mg are likely needed to cause mucosal reaction.  GI symptoms in patients with symptomatic celiac disease who adhere to a gluten-free diet typically resolve within a few weeks; these patients experience the normalization of nutritional measures, improved growth in height and weight (with resultant normal stature), and normalization of hematological and biochemical parameters.

Furthermore, treatment with a gluten-free diet reverses the decrease in bone mineralization and the risk for fractures . Symptomatic children treated with a gluten-free also improve their sense of physical and psychological well being.

For a long time, oats were considered toxic as well, and their elimination from the diet had been recommended. However, over the past decade, a growing body of scientific evidence obtained from in vitro studies as well as from clinical investigations (particularly in adults but also, more recently, in children) suggests that oats are totally safe. Because of uncontrolled harvesting and milling procedures, as well as the possibility that lines of manufacturing used for wheat-based flours are also used in the preparation of oat-based foods, cross-contamination of oats with gluten is still a concern.

Lactose is often eliminated in the initial phases of dietary treatment as well. This is because lactase deficiency is thought to accompany the flat mucosa. However, most newly diagnosed patients with celiac disease are diagnosed in the absence of overt malabsorptive symptoms; in these circumstances, clinically significant lactose malabsorption or intolerance is rarely seen. Furthermore, even in cases with obvious malabsorption, the recovery of lactase activity is typically fast; thus, a lactose-free diet must be used on a short-term basis only, even in these individuals.

The American Dietetic Association (ADA) publishes guidelines for the dietary treatment of celiac disease. They are a reliable source of information for a gluten-free. However, because of the dynamics of this field, the diet requires ongoing collaboration between patients, health care providers, and dietitians.

Consultations

Because of the protean nature of celiac disease, multiple consultations may be necessary. For example, consultations with an endocrine specialist should be arranged for patients who also have Hashimoto thyroiditis or type I diabetes mellitus, and a rheumatologist must be consulted for patients who have arthritis.

Diet

Diet

At present, the only effective treatment is a life-long gluten-free diet.No medication exists that will prevent damage or prevent the body from attacking the gut when gluten is present. Strict adherence to the diet allows the intestines to heal, leading to resolution of all symptoms in most cases and, depending on how soon the diet is begun, can also eliminate the heightened risk of osteoporosis and intestinal cancer. Dietician input is generally requested to ensure the patient is aware which foods contain gluten, which foods are safe, and how to have a balanced diet despite the limitations. In many countries, gluten-free products are available on prescription and may be reimbursed by health insurance plans, however gluten-free products are hard to find in Asian countries because Coeliac Disease is not a serious epidemic in these countries. Websites such as glutenfreemall.com helps Americans as well as others order gluten-free products domestically and internationally. More manufacturers are producing gluten-free products, some of which are almost indistinguishable from their gluten-containing counterparts.

The diet can be cumbersome; failure to comply with the diet may cause relapse. The term gluten-free is generally used to indicate a supposed harmless level of gluten rather than a complete absence. The exact level at which gluten is harmless is uncertain and controversial. A recent systematic review tentatively concluded that consumption of less than 10 mg of gluten per day is unlikely to cause histological abnormalities, although it noted that few reliable studies had been done.[55] Regulation of the label gluten-free varies widely by country. For example, in the United States, the term gluten-free is not yet regulated.[56] The current international Codex Alimentarius standard, established in 1981, allows for 50 mg N/100 g on dry matter, although a proposal for a revised standard of 20 ppm in naturally gluten-free products and 200 ppm in products rendered gluten-free has been accepted. Gluten-free products are usually more expensive and harder to find than common gluten-containing foods. Since ready-made products often contain traces of gluten, some coeliacs may find it necessary to cook from scratch.

Even while on a diet, health-related quality of life (HRQOL) may be lower in people with coeliac disease. Studies in the United States have found that quality of life becomes comparable to the general population after staying on the diet, while studies in Europe have found that quality of life remains lower, although the surveys are not quite the same. Men tend to report more improvement than women. Some have persisting digestive symptoms or dermatitis herpetiformis, mouth ulcers, osteoporosis and resultant fractures. Symptoms suggestive of irritable bowel syndrome may be present, and there is an increased rate of anxiety, fatigue, dyspepsia and musculoskeletal pain.

Refractory disease

A tiny minority of patients suffer from refractory disease, which means they do not improve on a gluten-free diet. This may be because the disease has been present for so long that the intestines are no longer able to heal on diet alone, or because the patient is not adhering to the diet, or because the patient is consuming foods that are inadvertently contaminated with gluten. If alternative causes have been eliminated, steroids or immunosuppressants (such as azathioprine) may be considered in this scenario.[6]

Experimental treatments

Various other approaches are being studied that would reduce the need of dieting. All are still under development, and are not expected to be available to the general public for a while:

  • Genetically engineered wheat species, or wheat species that have been selectively bred to be minimally immunogenic. This, however, could interfere with the effects that gliadin has on the quality of dough.
  • A combination of enzymes (prolyl endopeptidase and a barley glutamine-specific cysteine endopeptidase (EP-B2)) that degrade the putative 33-mer peptide in the duodenum. This combination would enable coeliac disease patients to consume gluten-containing products.[63]
  • Inhibition of zonulin, an endogenous signalling protein linked to increased permeability of the bowel wall and hence increased presentation of gliadin to the immune system.[64]
  • Other treatments aimed at other well-understood steps in the pathogenesis of coeliac disease, such as the action of HLA-DQ2 or tissue transglutaminase and the MICA/NKG2D interaction that may be involved in the killing of enterocytes (bowel lining cells).

Activity

No additional restriction is necessary beyond that imposed by the patient’s fatigue. However, if a completely gluten-free diet is followed, celiac disease completely regresses, and individuals have a completely normal quality of life.

Consultations

  • Dietitian
  • Nutritionist

Diet

Complete elimination of gluten-containing grain products, which include wheat, rye, and barley, is essential to treatment. After an initial period of avoidance, oats might be reintroduced into the diet of patients with celiac disease. These patients should be monitored carefully for recurrent symptoms.

Medication

Glucocorticoids

Corticosteroids can rapidly control severe symptoms of celiac disease (CD). They may also have a role in rare cases in which the patient has no response to diet; this condition is known as refractory celiac disease and occurs exclusively in adults (1-3% of total).

For celiac disease in children, steroids are almost never needed.

Some cases of refractory celiac disease (with all other forms of colitis and enteritis excluded) respond to parenteral corticosteroids, for reasons unknown. Exclude other etiologies of failure to thrive, especially in children, because systemic steroids can pose risk to growth. Sodium succinate salt formulation may be administered IV or IM.

  • The primary treatment of celiac sprue is dietary.
    • Removal of gluten from the diet is essential. Complete avoidance of gluten-containing grain products is relatively difficult for patients to achieve and maintain because certain products, such as wheat flour, are virtually ubiquitous in the American diet.
    • Careful and extensive indoctrination of the patient by the physician and the dietitian is often necessary to achieve full compliance.
  • Corticosteroids
    • A small percentage of patients with celiac sprue fail to respond to a gluten-free diet. In some patients who are refractory, corticosteroids might be helpful.
    • In patients who fail to respond to corticosteroids, other comorbid conditions, such as lymphomas of the small intestine, have to be ruled out.

The goals of pharmacotherapy are to reduce morbidity and to prevent complications. Corticosteroids might be indicated in patients with refractory celiac sprue.

Corticosteroids have anti-inflammatory properties and cause profound and varied metabolic effects. These agents modify the body’s immune response to diverse stimuli.

Can be used in patients with refractory celiac sprue. Might decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

Adult

30-40 mg/d PO; taper off completely in 6-8 wk

Pediatric

1 mg/kg/d PO; not to exceed 30 mg/d; taper off completely in 6-8 wk

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