Down's syndrome is strongly associated with coeliac disease

Abstract

Background - There is evidence of an increased prevalence of coeliac disease in Down's syndrome. Aims - To investigate the association, patients with Down's syndrome and matched controls were examined. Methods - Fifty nine patients with Down's syndrome residing in government institutions in the Hunter region of New South Wales were studied. Four were excluded (terminally ill = 1, uncooperative = 3). Each of 55 patients was matched for age, sex, and residence with a control patient. Patients with both positive IgA and HgG antigliadin antibodies were considered for endoscopical duodenal biopsy. Results - Twenty one patients and two controls had raised IgA and IgG antibodies (χ2 = 19.4; p < 0.001). Tissue was obtained in 18 patients. Two had characteristic flat, five pronounced lymphocytic infiltration not diagnostic of coeliac disease, two giardiasis, and eight were normal. In one the tissue was not suitable for analysis. There were few differences between the subgroups in their anthropomorphic, biochemical, or haematological findings. Conclusions - The prevalence of coeliac disease in these 51 patients with Down's syndrome is at least two (3.9%; 95% confidence interval (95% CI) 0%-9.2%) and could be as many as seven (13.7%; 95% CI 4.3%-23.2%). In this community the prevalence of coeliac disease in Down's syndrome is increased more than 100-fold (x135-473).