Preface: The International Workshop on Celiac Disease and Malignancy

Abstract

Celiac disease is a permanent intolerance for certain proteins of wheat, rye, barley, and oats (gluten) characterized by villous atrophy of the small intestine. The disease is treated with a gluten-free diet (GFD). The incidence of clinical CD is approximately 1:2,000 live births, but subclinical CD has been found in about 1:300 schoolchildren. Moreover, CD may in general be a subclinical condition: a ratio of nearly 1:7 has been found for recognized versus unrecognized CD cases. The association between clinical CD and malignancies is well established: cancer develops in 8% to 13% of patients, but GFD plays a protective role against malignancy. In CD there is a 43-fold relative risk (RR) of non-Hodgkin small-bowel lymphoma, a 12-fold RR of cancer of the esophagus, and a 10-fold RR of cancer of the mouth and pharynx. Celiac patients have a higher incidence of non-Hodgkin lymphoma (NHL) and other associated malignancies than the general population. However, it is not known whether the risk of malignancy for patients with subclinical disease is the same as for patients with overt CD. If the prevalence of NHL is higher in untreated patients with subclinical CD, the identification and treatment of missed cases of CD should become relevant in the prevention of cancer. This publication contains the proceedings of an international workshop on CD and malignancy that took place September 21-22, 1995, at the Department of Paediatrics of the Leiden University Hospital, Leiden, the Netherlands (Fig. 1). During this workshop a group of specialists, actively involved in both care of patients and research, discussed the subject of CD and malignancy. This is the reason that this publication not only contains the abstracts of the lectures given by the invited speakers but also reports the discussions among the participants, which were recorded on video, with audio, during the meeting. The differences found in the incidence and prevalence of CD in different countries in Northern and Western Europe, and the possible mitigating factors, were discussed. However, it was concluded that in all the countries where serological screening for CD has been performed, a very consistent prevalence of gluten-sensitive enteropathy of 1/150 to 1/300 screened persons was found. The available data on CD and malignancy, including those coming from the group of patients with dermatitis herpetiformis, were reviewed. The possibility that CD may in fact be a pre-malignant, treatable disorder, the hypothetical immunogenetic mechanisms involved and the consequences for Public Health were discussed. Finally, during the workshop a research protocol was developed to evaluate the prevalence of untreated CD in subjects with NHL in a multicenter, prospective, case-control study. At the moment a number of investigators from different countries, most of them present at the workshop, are working in a collaborative effort to study the relationship between CD and malignancy.FIG. 1: . Some of the workshop participants.