Mortality and Malignancy in Celiac Disease

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Introduction

Celiac disease (CD) is a chronic immune-mediated disease characterized by inflammation and villous atrophy (VA) in the small intestine.1 It is found in people worldwide, occurring in about 1% to 2% of the Western population.2, 3

Many patients with CD remain undiagnosed for several years before receiving a correct diagnosis and adequate treatment. Treatment consists of a lifelong gluten-free diet (GFD). Despite treatment with a GFD, most studies suggest that diagnosed CD is associated with increased mortality, as well as with certain types of cancer. Persistent inflammation with consequent malabsorption of micronutrients may be 1 explanation for this excess risk.

This article reviews a selection of studies on mortality and malignancy and describes how their results might influence the management of patients with CD.

Section snippets

Diagnosed CD

Although several studies indicate that diagnosed CD is associated with increased mortality, data are contradictory with regards to the extent of such excess mortality. The first half of this article reviews the largest and most recent mortality studies, all of which included at least 1000 patients with CD.4, 5, 6, 7, 8

The first such study was an Italian multicenter study of 1072 individuals with CD.8 In this study, adults with biopsy-proven CD from 11 gastroenterology units were identified (

Any Malignancy

Many studies on CD and malignancy typically identified patients with CD at 1 or a small number of referral centers, potentially leading to selection bias and overinflated risk estimates. Other studies have been limited by small number of cancers, leading to wide CIs and uncertainty regarding true risk estimates, whereas some older studies also included individuals with cancer diagnosed before or at the same time as CD or only examined death from cancer. Together, such limitations may lead to an

Implications for screening

Several studies noted in this paper on mortality and malignancy in CD shed light on the debate over screening for CD in the general population, and specifically argue against screening. Corrao and colleagues8 reported that mortality was increased only in symptomatic individuals, the consequence being that asymptomatic patients with undiagnosed CD are unlikely to benefit from a CD diagnosis in relation to mortality risk. Recent data suggest that individuals with dermatitis herpetiformis, which

Summary

It seems that patients with diagnosed CD are at a small increased risk of mortality. With the exception of an increased risk of LPM and a modest, short-term excess risk of GI malignancy, patients with CD do not seem to be at increased risk of malignancy. Future research should focus on the importance of dietary adherence to reduce existing risks, identify other risk factors for mortality and LPM in patients with CD, and evaluate the risk for mortality and malignancy in individuals with

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    Disclosure/conflict of interest declaration: None.

    Grant support (funding): JFL was supported by grants from the Swedish Society of Medicine, the Swedish Research Council – Medicine (522-2A09-195), the Swedish Celiac Society, and the Fulbright commission.

    Independence (role of the sponsors): None of the funders had any role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; and preparation, review, or approval of the article.

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