Bone involvement in adult patients affected with Ehlers-Danlos syndrome.
Abstract
Unlabelled 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 Conclusions EDS patients have reduced BMD and…
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The 2017 diagnostic criteria did not highlight some extra-articular manifestations of hEDS such as bone involvement, neurologic involvement, sleep disorders, and immune system disorders.
“and did not highlight some extra-articular manifestations of hEDS such as bone involvement [18], neurologic involvement (including small-fiber neuropathy [19]), sleep disorders [20], and immune system disorders such as mast cell disorders [21].”
From Are patients with hypermobile Ehlers–Danlos syndrome or hypermobility spectrum disorder so different? (2021) · cites this paper as [18]
None of the prior studies suggested a difference between hEDS and HSD patients, but a high prevalence of bone involvement seems confirmed.
“None of these prior studies suggested a difference between hEDS and HSD patients, but a high prevalence of bone involvement seems confirmed.”
From Are patients with hypermobile Ehlers–Danlos syndrome or hypermobility spectrum disorder so different? (2021) · cites this paper as [18]
Cites
The Ehlers–Danlos syndrome, a disorder with many faces
De Paepe, A., & Malfait, F. (2012) · Clinical Genetics · doi:10.1111/j.1399-0004.2012.01858.x · Cited by 379 · see citing works
Ehlers-Danlos syndromes and Marfan syndrome
Callewaert, B., Malfait, F., Loeys, B., & De Paepe, A. (2008) · Best Practice & Research Clinical Rheumatology · doi:10.1016/j.berh.2007.12.005 · Cited by 257 · see citing works
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Aubry-Rozier, B., Schwitzguebel, A., Valerio, F., Tanniger, J., Paquier, C., Berna, C., Hügle, T., & Benaim, C. (2021) · Rheumatology International · doi:10.1007/s00296-021-04968-3 · Cited by 66 · see citing works
- Authors
- Eller-Vainicher, C., Bassotti, A., Imeraj, A., Cairoli, E., Ulivieri, F. M., Cortini, F., Dubini, M., Marinelli, B., Spada, A., & Chiodini, I.
- Affiliations
- Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico (IT)
- University of Milan (IT)
- Corresponding author
- Chiodini, I.
- Venue
- Osteoporosis International
- Published
- 2016-04-15
- Volume
- 27
- Issue
- 8
- Pages
- 2525-2531
- ISSN
- 0937-941X (ISSN-L)
- 1433-2965
- OpenAlex
- W2338844665
- Cited by
- 51
- References
- 2
- Citations by year
- 2026: 5 · 2025: 3 · 2024: 4 · 2023: 3 · 2022: 6 · 2021: 8 · 2020: 5 · 2019: 6 · 2018: 7 · 2017: 3 · 2016: 1
- FWCI
- 4.57
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Osteoporosis International (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Health Sciences › Medicine › Orthopedics and Sports Medicine › Bone health and osteoporosis research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Dermatological and Skeletal Disorders
- Keywords
- Ehlers-Danlos syndrome, bone mineral density, bone quality, trabecular bone score, vertebral fractures, hypermobility Ehlers-Danlos syndrome, classical Ehlers-Danlos syndrome, connective tissue diseases, bone health, bone turnover, vitamin D, calcium and phosphorus metabolism, lumbar spine, femoral neck, adults
- MeSH terms
- Adult, Ehlers-Danlos Syndrome, Female, Femur Neck, Humans, Lumbar Vertebrae, Male, Middle Aged, Absorptiometry, Photon, Bone Density, Spinal Fractures, Bone Remodeling
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