Concise reports
Assessment of bone in Ehlers Danlos syndrome by ultrasound and
densitometry
Department of Rheumatology UMDS Guys and St Thomas's
Hospital, London
Correspondence to: Dr AL Dolan, Department of Rheumatology, Greenwich District Hospital, Vanburgh Hill, London SE10 9EH.
Accepted for publication 21 August 1998
OBJECTIVE
Ehlers Danlos syndrome (EDS) is an
inherited disorder of connective tissue characterised by
hyperextensible skin, joint laxity, and easy bruising. There are
phenotypic similarities with osteogenesis imperfecta, but in EDS a
tendency to fracture or altered bone mass has not previously been
considered to be a cardinal feature.
METHODThis case-control design study investigates
whether 23 patients with EDS had differences in fracture rates, bone
mass, and calcaneal ultrasound parameters compared with age and sex
matched controls.
RESULTS23 cases of EDS (mean (SD) age 38.5 (15.5)) were compared with 23 controls (mean age 37.8 (14.5)). A
significant reduction in bone density measured by dual energy
x ray absorptiometry was found at the neck of femur by 0.9 SD, p = 0.05, and lumbar spine by 0.74 SD, p = 0.02. At the calcaneum,
broad band ultrasound attenuation and speed of sound were significantly
reduced compared with controls by 0.95 SD (p = 0.004) and 0.49 SD (p = 0.004) for broad band ultrasound attenuation and speed of sound
respectively. Broad band ultrasound attenuation and speed of sound
remained significantly reduced after adjusting for bone mineral density (BMD). After adjusting for functional status (HAQ), age and sex, hypermobility was inversely correlated with broad band ultrasound attenuation and SOS, but not BMD at hip or spine. Previous fracture was
10 times more common in EDS (p < 0.001), with 86.9% of patients reporting a total of 47 low impact fractures, compared with 8.7% of controls.
CONCLUSIONThis study has identified a tendency of
EDS patients to fracture, have low bone mass and abnormal bone
structure. The aetiology is likely to be multifactorial, with an
inherited structural element, accentuated by immobility or reduced
exercise. This is one of the first clinical studies to suggest
ultrasound can detect structural differences in bone, independent of
dual energy x ray absorptiometry.
© 1998 by Annals of the Rheumatic Diseases
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