Nationwide population-based cohort study of celiac disease and risk of Ehlers-Danlos syndrome and joint hypermobility syndrome

Abstract Background Patients with celiac disease (CD) often have articular complaints, and small prior studies suggest an association with Ehlers-Danlos syndrome (EDS)/joint hypermobility syndrome (JHS). Aims This study examines the risks of EDS/JHS in patients with CD. Methods This cohort study compared all individuals in Sweden diagnosed with CD based on small intestinal biopsy between 1969–2008 ( n  = 28,631) to 139,832 matched reference individuals, and to a second reference group undergoing biopsy without having CD ( n  = 16,104). Rates of EDS/JHS were determined based on diagnostic codes in the Swedish Patient Register. Hazard ratios (HRs) for EDS/JHS were estimated through Cox regression. Results There are 45 and 148 cases of EDS/JHS in patients with CD and reference individuals, respectively. This corresponds to a 49% increased risk of EDS/JHS in CD (95%CI = 1.07–2.07). The HR for EDS was 2.43 (95%CI = 1.20–4.91) and for JHS 1.34 (95%CI = 0.93–1.95). Compared to reference individuals undergoing intestinal biopsy, CD was not a risk factor for EDS/JHS. A stronger association was seen in patients initially diagnosed with EDS/JHS and subsequently diagnosed with CD (odds ratio = 2.29; 95%CI = 1.21–4.34). Conclusions Individuals with CD have higher risk of EDS/JHS than the general population, which may be due to surveillance bias or factors intrinsic to celiac development.