Background Refractory coeliac disease (RCD) is a rare but serious complication of coeliac disease and is characterised by non-responsiveness to a gluten free diet in the presence of a clonal population of T lymphocytes within the small intestine. The risk of progression from RCD to enteropathy associated T cell lymphoma is estimated at 60–80% and is associated with a poor survival.
Purpose Therapeutic options for RCD are limited. Immunosuppression with corticosteroids, thiopurines and infliximab have been used but promote the progression to lymphoma.
Material and methods A 72-year-old woman with a 4 year history of coeliac disease was studied. She was treated initially with a gluten free diet and pharmacological treatment with azathioprine and oral corticosteroids. Some improvement in her symptoms was observed but this was not sustained. Therefore, infliximab treatment 5 mg/kg every 8 weeks was initiated.
Results In February 2014, the patient began to receive therapy with infliximab. Response to treatment after 6 months was partial, maintaining a weight of 47 kg without diarrhoea, although she continued to have hypoalbuminaemia–cholesterolaemia and anaemia. In August 2014, infliximab treatment was stopped owing to surgical intervention. In September 2014, the patient weighed 37 kg and was admitted to hospital with severe diarrhoea. During her hospital stay, infliximab treatment was restarted. In April 2015, the patient weighed 50 kg and blood test levels were in the normal range. In September 2015, the patient suffered musculoskeletal pains, mild fever (37.5°C) and weight loss of 5 kg. On 3 December/2015, the patient received an infliximab dose, after which she had dark urine, musculoskeletal pains, chills and diarrhoea. On 17 December/2015, the patient was admitted to hospital because of deterioration in her general health, fever, musculoskeletal pains which impeded walking and anti-infliximab antibodies levels of 2.27. In the absence of a response, infliximab was stopped. On 4 January 2016, CT scan of the abdomen/thorax was performed and metastatic pericardium, bone and pancreatic disease was observed.
On 12 January 2016, a biopsy was performed which determined ALK negative anaplastic large cell lymphoma. On 25 January 2016 the patient’s condition aggravated and she died as a result of multiple organ failure caused by lymphoma.
Conclusion Infliximab is an effective treatment that may be considered in a small number of patients with refractory coeliac disease, resistant to other therapies due to the increased risk of lymphoma risk.
No conflict of interest
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