Higher fracture prevalence and smaller bone size in patients with hEDS/HSD—a prospective cohort study

• Published in: Osteoporosis international 2020-05, Vol.31 (5), p.849-856
• Main Authors: Banica, T., Coussens, M., Verroken, C., Calders, P., De Wandele, I., Malfait, F., Zmierczak, H.-G., Goemaere, S., Lapauw, B., Rombaut, L.
• Format: Article
• Language: English
• Subjects: Adult; Body composition; Bone composition; Bone Density; Bone loss; Bone mineral content; Bone mineral density; Bone strength; Cohort analysis; Collagen; Computed tomography; Connective tissues; Cortical bone; Dual energy X-ray absorptiometry; Ehlers-Danlos Syndrome; Endocrinology; Female; Fractures; Fractures, Bone; Health risk assessment; Humans; Mechanical loading; Medicine; Medicine & Public Health; Middle Aged; Original Article; Orthopedics; Osteoporosis; Prevalence; Prospective Studies; Rheumatology
• ISSN: 0937-941X; 1433-2965
• DOI: 10.1007/s00198-019-05269-z

Summary 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 control subjects (all p