BackgroundThe association between Down syndrome and celiac disease has been reported by many studies. However, the prevalence of celiac disease (CD) in Down syndrome (DS) varies considerably across studies (from 0 % to 19 %). The aim of this study was to use meta-analysis to exam the prevalence of CD in patients with DS.MethodsA systematic search of English articles from Pubmed, Web of Science and CNKI without year limitation. Data were extracted by two independent observers and pooled using a random effects model by the Comprehensive Meta-Analysis Version 2 software.ResultsA pooled analysis, based on 31 studies included 4383 individuals, revealed prevalence of biopsy-confirmed CD of 5.8 % (95 % CI = 4.7-7.2 %) in patients with DS. Sub-group analysis showed a slightly higher prevalence of CD in children with DS (6.6 %; 17 studies), than in age mixed samples with both children and adults (5.1 %; 13 studies). In addition, most of the studies included in this meta-analysis were from Europe and America, with the prevalence of celiac disease of 6 % (21 studies) and 5.7 % (6 studies) in DS patients, respectively. Furthermore, meta-regression analysis suggested that proportion of antibody-positive individuals that underwent small intestine biopsy had moderating effect on the outcome of the meta-analysis.ConclusionsThese results demonstrated that patients (children) with Down syndrome had high prevalence of CD (more than one in twenty). The prevalence is high enough to motivate screening CD in DS children.