Higher fracture prevalence and smaller bone size in patients with hEDS/HSD—a prospective cohort study
Abstract
Increased fracture risk in patients with Ehlers-Danlos syndromes has been reported, but the reasons for it are incompletely understood. We aimed to investigate possible determinants of this increased risk and found that hEDS/HSD patients present with a cortical bone size deficit compared with control subjects, possibly related to lower mechanical loading. Introduction The Ehlers-Danlos syndromes (EDS) comprise a group of heritable connective tissue disorders caused by defects in the biosynthesis, secretion, and/or organization of fibrillar collagens which might impair bone strength. Our aim was to compare fracture prevalence, volumetric and areal bone mineral density (BMD), bone geometry, muscle size and the muscle-bone interaction, body composition and longitudinal changes therein between patients with hypermobile EDS (hEDS) or hypermobility spectrum disorder (HSD), and healthy control subjects. Methods Cross-sectional data comprised 39 female hEDS/HSD patients (age 41 ± 11 years) and 43 age-matched controls. After 8 years, 27 hEDS/HSD and 17 control subjects were re-evaluated. Tibial trabecular and cortical volumetric BMD, bone mineral content (BMC), cortical bone geometry, and lower leg muscle cross-sectional area (CSA) were measured using pQCT. Body composition, areal BMD, and BMC were determined by DXA. Results At baseline, patients with hEDS/HSD presented with a smaller cortical bone area, smaller cortical thickness and muscle CSA, and a higher fracture prevalence than…
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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 [35]
Cites
The 2017 international classification of the Ehlers–Danlos syndromes
Malfait, F., Francomano, C., Byers, P., Belmont, J., Berglund, B., Black, J., Bloom, L., Bowen, J. M., Brady, A. F., Burrows, N. P., et al. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31552 · Cited by 1,936 · see citing works
Self-citationA framework for the classification of joint hypermobility and related conditions
Castori, M., Tinkle, B., Levy, H., Grahame, R., Malfait, F., & Hakim, A. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31539 · Cited by 602 · see citing works
Self-citationThe 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
Self-citationRombaut, L., Malfait, F., Cools, A., De Paepe, A., & Calders, P. (2010) · Disability and Rehabilitation · doi:10.3109/09638280903514739 · Cited by 236 · see citing works
Self-citationBone geometry and skeletal fragility
Bouxsein, M. L., & Karasik, D. (2006) · Current Osteoporosis Reports · doi:10.1007/s11914-006-0002-9 · Cited by 165 · 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
“[35] suggested that bone fragility in hEDS or HSD patients could be linked to lower mechanical strain.”
BackgroundPain in the Ehlers–Danlos syndromes: Mechanisms, models, and challenges
Malfait, F., Colman, M., Vroman, R., De Wandele, I., Rombaut, L., Miller, R. E., Malfait, A., & Syx, D. (2021) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31950 · Cited by 41 · see citing works
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- Authors
- Banica, T., Coussens, M., Verroken, C., Calders, P., De Wandele, I., Malfait, F., Zmierczak, H.-G., Goemaere, S., Lapauw, B., & Rombaut, L.
- Affiliations
- Ghent University Hospital (BE)
- Ghent University (BE)
- Corresponding author
- Banica, T.
- Venue
- Osteoporosis International
- Published
- 2019-12-24
- Volume
- 31
- Issue
- 5
- Pages
- 849-856
- ISSN
- 0937-941X (ISSN-L)
- 1433-2965
- OpenAlex
- W2996061948
- Cited by
- 19
- References
- 5
- Citations by year
- 2026: 1 · 2025: 2 · 2024: 4 · 2023: 1 · 2022: 4 · 2021: 4 · 2020: 3
- FWCI
- 1.65
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Osteoporosis International (publishedVersion)
- PubMed (publishedVersion)
- Ghent University Academic Bibliography (Ghent University) (publishedVersion)
- Ghent University Academic Bibliography (Ghent University) (submittedVersion)
- Funders
- Universiteit Gent (BOFMET2015000401)
- 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
- hypermobile Ehlers-Danlos syndrome, hypermobility spectrum disorders, fracture risk, cortical bone thickness, bone geometry, muscle cross-sectional area, muscle-bone interaction, mechanical loading, bone mineral density, body composition, connective tissue diseases, collagen defects
- MeSH terms
- Adult, Ehlers-Danlos Syndrome, Female, Humans, Middle Aged, Prospective Studies, Bone Density, Prevalence, Fractures, Bone
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