Approach

The only accepted treatment of celiac disease is a strict lifelong gluten-free diet.

Dietary advice

The diet should not be started until definitive diagnosis has been made by small intestinal histology.

After diagnosis, the patient should be referred to a dietitian with specific training in celiac disease and the gluten-free diet. Dietary counseling is important because the gluten-free diet has been associated with lower intake of fiber, as well as vitamin and micronutrient deficiencies, and a higher intake of calories, simple carbohydrates, and saturated fats.[62][99] Celiac disease patients are at risk of becoming overweight/obese.[100]

Quality of life for celiac patients has been shown to improve with adherence to a gluten-free diet.[101] However, gluten-free diet adherence is difficult, with dietary lapses in the majority of patients.[102] The importance of the diet should be stressed, and social support evaluated and encouraged within the family and by membership in celiac disease advocacy groups.

Supplementation

Patients should be checked for common deficiencies including iron, vitamin D, vitamin B12, and folate. All patients with celiac disease should be recommended to take calcium and vitamin D supplements. Iron should only be given to individuals with iron deficiency. Vitamin B12 (cyanocobalamin) and folate deficiencies should be corrected, especially since the gluten-free diet may be low in folate. 

Bone mineral density should be evaluated after approximately 1 year on a gluten-free diet to assess for osteopenia or osteoporosis. 

Failure to respond to treatment

For individuals who do not respond to a gluten-free diet, the most common problem is continued gluten exposure. There is evidence that, on a supposedly adequate gluten-free diet, patients consume enough gluten to trigger symptoms.[103][104]

The initial step in the evaluation should be repeating immunoglobulin A-tissue transglutaminase (IgA-tTG) titer and referral to a dietitian with expertise in celiac disease. If there is no evidence of continuing gluten intake, referral to a gastroenterologist with experience in the evaluation of nonresponsive celiac disease is recommended. While gluten exposure is the most common cause of nonresponsive celiac disease, many other conditions can explain symptoms, such as irritable bowel syndrome, other food intolerances, microscopic colitis, or small intestinal bacterial overgrowth.[105][106]

Although positive IgA-tTG is indicative of intestinal injury and gluten exposure, a negative value cannot exclude continued intestinal injury.[107][108] If symptoms persist or relapse without an alternative explanation, repeat esophagogastroduodenoscopy and duodenal biopsies should be performed regardless of serologic titers.[108]

Refractory celiac disease

Refractory celiac disease is defined as the persistence of malabsorption symptoms and villous atrophy despite strict gluten withdrawal for 12 months and no evidence of another abnormality including overt lymphoma. It is present in <1% of patients with celiac disease, and may be a spectrum determined by T-cell clonality and loss of normal intraepithelial cell markers.[109] Common associations with refractory celiac disease include ulcerative jejunitis and enteropathy-associated T-cell lymphoma. The outlook for patients is generally poor. They should be cared for at a center experienced in celiac disease.

Celiac crisis

Celiac crisis is rare and presents with hypovolemia, severe watery diarrhea, acidosis, hypocalcemia, and hypoalbuminemia. Patients are often emaciated and have nutritional deficiencies caused by longstanding, untreated celiac disease. In addition to rehydration and correction of electrolyte abnormalities, these few patients may benefit from a short course of systemic glucocorticoid therapy until the gluten-free diet takes effect.

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