Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

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celiac disease

1st line – gluten-free diet

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celiac disease
1st line – 

gluten-free diet

The lifelong gluten-free diet is the only accepted treatment of celiac disease. Consultation with a dietitian should be sought because: adherence is difficult; dietary changes may lead to deficiencies of fiber and other nutrients; the gluten-free diet can involve a higher intake of calories, simple carbohydrates, and saturated fats.[94][139]​Patients with celiac disease are at risk of becoming overweight/obese.[140]

Although a small percentage of people may react to avenin or cross-contamination with other gluten-containing cereals, gluten-free oats are recommended in the diet for their nutrition benefits (soluble fiber, polyunsaturated oil, vitamin B complex, and iron).[70]

Quality of life for patients with celiac disease has been shown to improve, but not normalize, with adherence to a gluten-free diet.[141] Gluten-free diet adherence is difficult, with dietary lapses in the majority of patients.[142] The importance of the diet should be stressed, and social support should be evaluated and encouraged within the family and by membership in celiac disease advocacy groups. 

There is substantial evidence that oats that are not contaminated by wheat or barley are safe for the vast majority of patients with celiac disease.[156][157][158]​ Some patients may, however, be sensitive.[159] The American College of Gastroenterology guidelines recommend inclusion of gluten-free oats in diets of patients with celiac disease.[70]

In North America, some food manufacturers operate under a "Purity Protocol", which involves harvesting, transporting, storing, processing and manufacturing oats using processes that minimize the presence of gluten.[160] Oats are not recommended as part of a gluten-free diet in some countries, and local guidance should be consulted before recommending them.

Plus – vitamin and mineral supplementation

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celiac disease
Plus – 

vitamin and mineral supplementation

Treatment recommended for ALL patients in selected patient group

Patients should be checked for common deficiencies including iron, vitamin D, vitamin B12, and folate. Deficiencies of these vitamins and minerals are more common in people with celiac disease, compared with the general population.[143]

All patients with celiac disease should 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.

See Iron deficiency anemia, Vitamin D deficiency, Vitamin B12 deficiency, and Folate deficiency.

Bone mineral density evaluation is indicated in patients with celiac disease to assess for osteopenia or osteoporosis, but evidence regarding the optimal timing is scant. In individuals with other risk factors for osteoporosis, ages >50 years, with severe villous atrophy, a bone mineral density analysis at the time of diagnosis is indicated.[34]​ Some guidelines recommend evaluation of bone mineral density either at diagnosis or after 1 year on a gluten-free diet, as studies show that the bone density may improve on a gluten-free diet.[70][144]​​​​ Others recommend evaluation no later than ages 30-35 years, considering evidence showing a high rate of osteopenia in this population of patients with celiac disease.​[34][145]

Doses are individualized according to age and presence of deficiencies or decreased bone density.

Primary options

ergocalciferol (vitamin D2): 1000-2000 units orally once daily

and

calcium carbonate: 1000-1500 mg/day orally given in 3-4 divided doses

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OR

ergocalciferol (vitamin D2): 1000-2000 units orally once daily

and

calcium carbonate: 1000-1500 mg/day orally given in 3-4 divided doses

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and

ferrous sulfate: 300 mg orally (immediate-release) two to four times daily

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OR

cyanocobalamin (vitamin B12): 1000-2000 micrograms orally once daily for 1-2 weeks, followed by 500-1000 micrograms once daily; 1000 micrograms intramuscularly/subcutaneously once daily for 1 week, followed by 1000 micrograms once weekly for 1-2 months, then 1000 micrograms once monthly

OR

cyanocobalamin (vitamin B12) nasal: 500 micrograms into one nostril once weekly

OR

folic acid (vitamin B9): 0.4 to 0.6 mg orally once daily

failure to respond to therapy/refractory celiac disease

1st line – referral to dietitian or gastroenterologist

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failure to respond to therapy/refractory celiac disease
1st line – 

referral to dietitian or gastroenterologist

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.[146][147]

The initial step in the evaluation should be repeating immunoglobulin A-tissue transglutaminase 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.

If symptoms persist or relapse without an alternative explanation, repeat esophagogastroduodenoscopy and duodenal biopsies should be performed regardless of serologic titers.[151]

A first-line therapy in these patients in some centers is open-capsule budesonide.[161]

The outlook for patients with refractory celiac disease can be poor. They should be cared for at a center experienced in celiac disease.

Primary options

budesonide: 3 mg orally (delayed-release) three times daily

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celiac crisis

1st line – rehydration + correction of electrolyte abnormalities

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celiac crisis
1st line – 

rehydration + correction of electrolyte abnormalities

Celiac crisis is rare and presents with hypovolemia, severe watery diarrhea, acidosis, hypocalcemia, and hypoalbuminemia. Patients may have a precipitating major medical event, for example, recent abdominal surgery.[153] Cases have been reported in adults and children.​[153][154]​​ Patients require parenteral fluid replacement and nutritional support.[153]

Consider – corticosteroid

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celiac crisis
Consider – 

corticosteroid

Treatment recommended for SOME patients in selected patient group

In addition to rehydration and correction of electrolyte abnormalities, patients with celiac crisis may benefit from a short course of glucocorticoid therapy until the gluten-free diet takes effect.[155][153]

If patients are able to take oral medications, budesonide may be used initially. If this is not effective, prednisone or an equivalent systemic corticosteroid can be started, and should be tapered slowly after the patient is able to maintain hydration and nutritional status without intravenous supplementation.

Primary options

budesonide: 3 mg orally (delayed-release) three times daily

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OR

prednisone: 40-60 mg orally once daily initially then taper dose slowly

Secondary options

methylprednisolone sodium succinate: consult specialist for guidance on dose

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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