Delayed Diagnosis of Coeliac Disease Increases Cancer Risk

Marco Silano; Umberto Volta; Anna Maria Mecchia; Mariarita Dessì; Rita Di Benedetto; Massimo De Vincenzi; for the collaborating centers of the Italian registry of the complications of coeliac disease


BMC Gastroenterol 

In This Article

Abstract and Introduction

Background: The association between coeliac disease (CD) and neoplasms has been long established, but few data are available about the risk factors. The aim of this paper is to estimate the risk of developing a neoplasm among non diagnosed coeliac patients and to evaluate if this risk correlates with the age of patients at diagnosis of coeliac disease.
Methods: The study population consists of patients (n = 1968) diagnosed with CD at 20 Italian gastroenterology referral Centers between 1st January 1982 and 31st March 2005.
Results: The SIR for all cancers resulted to be 1.3; 95% CI = 1.0-1.7 p < 0.001. The specific SIRs for non Hodgkin lymphoma was 4.7; 95% CI = 2.9-7.3 p < 0.001, for the small bowel carcinoma 25; 95% CI = 8.5-51.4 p < 0.001, for non Hodgkin lymphoma 10; 95% CI = 2.7-25 p = 0.01, finally for the stomach carcinoma 3; 95% CI = 1.3-4.9 p < 0.08. The mean age at diagnosis of CD of patients that developed sooner or later a neoplasm was 47,6 ± 10.2 years versus 28.6 ± 18.2 years of patients who did not.
Conclusion: Coeliac patients have an increased risk of developing cancer in relation to the age of diagnosis of CD. This risk results higher for malignancies of the gastro-intestinal sites. An accurate screening for tumors should be performed in patients diagnosed with CD in adulthood and in advancing age.

Coeliac disease (CD) is a permanent autoimmune enteropathy triggered by the ingestion of gluten, the storage protein complex of wheat, rye and barley. Gluten causes, in genetically determined individuals carrying the HLA-DQ2/DQ8 haplotype, an inflammatory response of the small bowel mucosa, resulting in villous atrophy, infiltration of T-lymphocytes and hyperplasia of the cripts. The only known treatment for this disorder is the life - long withdrawn from the diet of the above mentioned cereals. CD occurs worldwide and its prevalence is estimated about 1/150 individuals.[1,2,3]

The association between CD and neoplasms has been long established.[4] In the '60s, a population-based study has already reported a 100-fold increased risk of non-Hodgkin's lymphoma in patients affected by CD.[5] More recently, this risk has been resized to 3-fold by an Italian study and 9-fold by a study from States.[6,7]

It has been also noted that CD patients have a higher risk of developing a small bowel adenocarcinoma respect to the general population and this neoplasm is the second invasive malignancy for incidence in these patients.[7,8,9,10]

In contrast, several studies have shown a lower risk of breast cancer in patients affected by CD.[11,12,13]

An increased mortality due to cancer in patients with CD has been also described.[14,15] There are considerable, but not definitive, evidences that the strict compliance to gluten-free diet is protective against the development of malignancies.[16,17,18]

We carried out a perspective, population-based study on 1968 coeliac patients with the aim to evaluate the malignancy risk of developing a malignancy and to assess if the delayed diagnosis of coeliac disease and the consequent prolonged dietary exposure to gluten increase the risk of developing a neoplasm.


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