Approach

The only accepted treatment of coeliac 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 coeliac disease and the gluten-free diet. Dietary counselling is important, beyond the gluten-free diet education, because a gluten-free diet has been associated with lower intake of fibre, as well as vitamin and micronutrient deficiencies, and a higher intake of calories, simple carbohydrates, and saturated fats.[94][131]​ Patients with coeliac disease are at risk of becoming overweight/obese.[132]

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 fibre, polyunsaturated oil, vitamin B complex, and iron).[73]

Quality of life for patients with coeliac disease has been shown to improve, but not normalise, with adherence to a gluten-free diet.[133] Gluten-free diet adherence is difficult, with dietary lapses in the majority of patients.[134] The importance of the diet should be stressed, and social support evaluated and encouraged within the family and by membership in coeliac disease advocacy groups.

Supplementation

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 coeliac disease, compared with the general population.[135] All patients with coeliac 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.

See:

Bone mineral density evaluation is indicated in patients with coeliac disease to assess for osteopenia or osteoporosis, but evidence regarding the optimal timing isare scant. In individuals with other risk factors for osteoporosis, aged >50 years, with severe villous atrophy, a bone mineral density analysis at the time of diagnosis is indicated. Some guidelines recommend evaluation of bone 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.[73][136]​​ Others recommend evaluation no later than age 30-35 years, considering evidence showing a high rate of osteopenia in this population of patients with coeliac disease.​[34][137]

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.[138][139]

The initial step in the evaluation should be repeating immunoglobulin A-tissue transglutaminase (IgA-tTG) titre and referral to a dietitian with expertise in coeliac disease. If there is no evidence of continuing gluten intake, referral to a gastroenterologist with experience in the evaluation of non-responsive coeliac disease is recommended. While gluten exposure is the most common cause of non-responsive coeliac disease, many other conditions can explain symptoms, such as irritable bowel syndrome, other food intolerances, microscopic colitis, or small intestinal bacterial overgrowth.[140][141]

Although positive IgA-tTG is indicative of intestinal injury and gluten exposure, a negative value cannot exclude continued intestinal injury.[142][143] If symptoms persist or relapse without an alternative explanation, repeat oesophagogastroduodenoscopy and duodenal biopsies should be performed, regardless of serological titres.[143]

Refractory coeliac disease

Refractory coeliac 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. A subgroup of patients with refractory coeliac disease develop complications of ulcerative jejunitis or enteropathy-associated T-cell lymphoma.[144] The outlook for patients is generally poor. They should be cared for at a centre experienced in coeliac disease.

Coeliac crisis

Coeliac crisis is rare and presents with hypovolaemia, severe watery diarrhoea, acidosis, hypocalcaemia, and hypoalbuminaemia. Patients may have a precipitating major medical event, for example, recent abdominal surgery.[145] Cases have been reported in adults and children.[145][146]​​​ In addition to parenteral fluid replacement, nutritional support, and correction of electrolyte abnormalities, most patients may benefit from a short course of systemic glucocorticoid therapy until the gluten-free diet takes effect.​[145][147]​​ If patients are able to take oral medications, budesonide may be used initially. If this is not effective, prednisolone 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.

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