Coeliac disease

Coeliac disease (CD) is a gluten dependent enteropathy with a very high prevalence¹ and an increased mortality rate.² Our knowledge regarding the clinical and pathogenetic aspects of CD has increased considerably over the past few years, but its diagnosis today—like several decades ago—is still based on the biopsy confirmed presence of duodenal–jejunal mucosal lesions that improve after a gluten free diet.

Although the greatest diagnostic challenge in CD concerns the identification of patients to be subjected to intestinal biopsy, rather than the choice of histopathological criteria, it is believed that the currently used criteria³ are often the source of disagreement between pathologists and clinicians and, at times, of misdiagnosis for the patients.

Based on the dynamic development pattern of coeliac lesions and on the frequent finding of cases of CD with mild lesions, Marsh³ proposed a four stage grading, namely: (1) type 1 infiltrative lesions, characterised by normal mucosal architecture with an increased number of intraepithelial lymphocytes (IELs); (2) type 2 hyperplastic lesions, characterised by an increase in crypt depth without villous flattening; (3) type 3 destructive lesions, characterised by villous atrophy and crypt hypertrophy; and (4) type 4 hypoplastic lesions, characterised by villous atrophy with normal crypt height and IEL count. Oberhuber and colleagues⁴ subsequently proposed a new standardised report scheme, based on the Marsh classification, in which stage 3 was split further into 3a, 3b, and 3c, characterised by mild villous flattening, marked villous flattening, and completely flat mucosa, respectively. At present, the Marsh classification of intestinal coeliac …