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Fear of malignancy has been the driver of many of the recommendations and guidelines on the investigation and management of coeliac disease since the malignant complications of coeliac disease were first recognised in the 1960s.1 2 What these early papers variously called reticulum cell sarcoma, intestinal reticulosis or malignant histiocytosis is now labelled as an enteropathy-associated T cell lymphoma (EATL). Cases can present acutely with obstruction or perforation, or chronically with diarrhoea, abdominal pain and weight loss, or, if coeliac disease has been previously diagnosed, a clinical relapse after responding to treatment with a gluten-free diet (GFD). It has a poor prognosis, generally following an intractable downhill course, with fewer than 20% surviving for >3 years.3–5
Initial estimates suggested that the relative risk of EATL might be 40–100 times higher than in the general population and that 10% of patients with coeliac disease diagnosed over age 50 would be harbouring a lymphoma.6–8 Furthermore, in one of the most cited papers ever to appear in Gut, Holmes et al reported that the long-term follow-up of a cohort of 210 patients with coeliac disease showed that the risks of developing EATL were much lower in those taking a strict GFD.9 These findings have been widely used to justify the need for a strict GFD, although the term ‘strict’ was not clearly defined in their paper.
As larger studies have appeared, based on populations rather than hospital series, …
Competing interests: None.
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