Bone involvement in adult patients affected with Ehlers-Danlos syndrome.

C. Eller-Vainicher, A. Bassotti, A. Imeraj, E. Cairoli, F. M. Ulivieri, F. Cortini, M. Dubini, B. Marinelli, A. Spada, I. Chiodini

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Abstract

Summary: 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

Original languageEnglish
Pages (from-to)2525-2531
Number of pages7
JournalOsteoporosis International
Volume27
Issue number8
DOIs
Publication statusPublished - 2016

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Keywords

  • Bone quality
  • Ehlers-Danlos syndrome
  • Trabecular bone score
  • Vertebral fractures

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism

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