Refractory coeliac disease is a rare subtype of coeliac disease typified by a lack of resolution of symptoms despite 6-12 months of a strict gluten-free diet, and in the absence of other factors that may affect disease response or the presence of malignancy.
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Epidemiology
Precise figures for the prevalence and incidence of the refractory form of coeliac disease are lacking. Data from small cohort studies of patients with coeliac disease suggests that the prevalence in coeliac populations is no greater than 1.5% 1. Women are more likely to be affected, reflecting the predilection for coeliac disease itself in women 1. Most cases are seen in the over 50 years old age group, and it is very rare in the sub-30 years old group.
Clinical presentation
Diarrhoea, abdominal pain and weight loss are the most frequently seen symptoms in a refractory coeliac disease presentation. Other manifestations include sequelae of hypovitaminoses, anaemia and lethargy. Venous thromboembolism and concurrent additional autoimmune conditions are common.
Radiographic features
Gastrointestinal findings of low specificity, such as mural thickening of the small bowel or mesenteric nodal enlargement, may be found in half of refractory coeliac disease cases 1. Other appearances associated with coeliac disease, such as atrophy of the spleen or cavitating mesenteric lymph node syndrome, are rare in the refractory form.
Treatment and prognosis
Most treatment is experimental, and it is difficult to develop effective therapies when the condition is so rare.
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