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


OBJECTIVE
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.
Celiac Disease Center, Department of Medicine, Columbia University College of Physicians and Surgeons, New York, NY, USA.
Monika
M
Laszkowska
Celiac Disease Center, Department of Medicine, Columbia University College of Physicians and Surgeons, New York, NY, USA.
Abhik
A
Roy
Celiac Disease Center, Department of Medicine, Columbia University College of Physicians and Surgeons, New York, NY, USA; Department of Medical Epidemiology and Biostatistics, Karolinska Institutet, Stockholm, Sweden.
Benjamin
B
Lebwohl
Celiac Disease Center, Department of Medicine, Columbia University College of Physicians and Surgeons, New York, NY, USA.
Peter H R
PH
Green
Department of Pediatrics, Linköping, University Hospital, Linköping, Sweden.
Heléne E K
HE
Sundelin
Department of Medical Epidemiology and Biostatistics, Karolinska Institutet, Stockholm, Sweden; Department of Pediatrics, Örebro University Hospital, Örebro, Sweden; Division of Epidemiology and Public Health, School of Medicine, University of Nottingham, Nottingham, UK. Electronic address: jonasludvigsson@yahoo.com.
Jonas F
JF
Ludvigsson
D000293:Adolescent; D000328:Adult; D016022:Case-Control Studies; D002446:Celiac Disease / Q000150:complications* / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D004535:Ehlers-Danlos Syndrome / Q000453:epidemiology*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007421:Intestine, Small / Q000473:pathology*; D007593:Joint Instability / Q000453:epidemiology*; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D012307:Risk Factors; D011795:Surveys and Questionnaires; D013548:Sweden; D055815:Young Adult
Cohort study; Epidemiology; Gluten; Hypermobility syndrome