Celiac Disease - NIH Consensus Development Program - National ...

Incidence and Prevalence of Celiac Disease Alaa Rostom, M.D., M.Sc., FRCPC ContextCeliac disease (CD) appears to represent a spectrum of clinical features andpresentations. Although “classical” CD (i.e., fully developed gluten-induced villous atrophy andclassical features of intestinal malabsorption) is most commonly described, it appears that mostpatients have atypical CD (fully developed gluten-induced villous atrophy found in the setting ofanother presentation such as iron deficiency, osteoporosis, short stature, or infertility) or silentCD (fully developed gluten-induced villous atrophy discovered in an asymptomatic patient byserologic screening or perhaps an endoscopy for another reason). The true prevalence of CD isdifficult to estimate because of this variable presentation, particularly when many patients mayhave little or no symptoms. With this limitation in mind, there appear to be importantgeographical and ethnic differences in the reported prevalence of CD. The prevalence appearshighest in Celtic populations where estimates of 1:300 to 1:122 have been described. In NorthAmerica, the prevalence has been estimated to be 1:3000, but a recent American study found it tobe 1:105 among the general not-at-risk population suggesting that the disease isunderrecognized.Objectives(1) To conduct a systematic review of the prevalence and incidence of CD in NorthAmerican and Western European populations. (2) To assess differences in prevalence amongdifferent geographical regions/countries and in at-risk populations such as relatives and patientswith type I diabetes.Data SourcesA comprehensive literature search was conducted by the National Library of Medicine incollaboration with the University of Ottawa Evidence-Based Practice Center (UO-EPC). Thesearches were run in MEDLINE (1966 to Oct 2003) and EMBASE (1974 to Dec 2003)databases.Study Selection and Data ExtractionThis study was conducted using accepted systematic review methodology. Studyselection was performed by two independent reviewers using three levels of screening withincreasingly more strict criteria to ensure that all relevant articles were captured. Articles passingthe third level screen fulfilled all the inclusion/exclusion criteria, allowed actual extraction of theprevalence and/or incidence data, and did not have fatal methodological flaws. Articles wereexcluded if the studied population was non-North American or Western European; patients were57