The Ehlers-Danlos syndrome is characterized by abnormal connective tissue but 
bone involvement is debated. We found a reduced BMD and bone quality and 
increased prevalence of asymptomatic vertebral fractures in eugonadal patients 
with Ehlers-Danlos syndrome. These findings suggest the need of a bone health 
evaluation in these patients.
INTRODUCTION: The Ehlers-Danlos (EDS) syndrome is characterized by abnormalities 
of the connective tissue leading to ligamentous laxity and skin and tissue 
fragility. We evaluated the bone metabolism, bone mineral density (BMD) and bone 
quality (measured by trabecular bone score, TBS), and the prevalence of 
vertebral fractures (VFx) in a group of eugonadal adult EDS patients.
METHODS: Fifty consecutive Caucasian patients, aged 30-50 years (36 females, 14 
males) with classical or hypermobility EDS and 50 age-, gender-, and body mass 
index (BMI)-matched control subjects were enrolled. In all subjects' 
calcium-phosphorous metabolism, bone turnover, BMD at the lumbar spine (LS) and 
femur (femoral neck, FN and total femur, FT) and TBS by dual-energy X-ray 
absorptiometry, and the VFx presence by spine radiograph were assessed.
RESULTS: Patients showed reduced BMD (Z-scores LS -0.45 ± 1.00, FN -0.56 ± 1.01, 
FT -0.58 ± 0.92) and TBS (1.299 ± 0.111) and increased prevalence of 
morphometric VFx (32 %) than controls (Z-scores LS 0.09 ± 1.22, FN 0.01 ± 0.97, 
FT 0.08 ± 0.89; TBS 1.382 ± 0.176; VFx 8 %, p <0.05 for all comparisons), while 
vitamin D levels, calcium-phosphorous metabolism, and bone turnover were 
comparable. Fractured EDS patients showed lower TBS values than non-fractured 
ones (1.245 ± 0.138 vs 1.325 ± 0.086, p < 0.05), despite comparable BMD. In EDS 
patients, the VFx presence was significantly associated with TBS even after 
adjusting for sex, age, BMD, EDS type, and falls frequency.
CONCLUSIONS: EDS patients have reduced BMD and bone quality (as measured by TBS) 
and increased prevalence of VFx.