Bone Disease in Patients with Ehlers-Danlos Syndromes

Curr Osteoporos Rep. 2020 Apr;18(2):95-102. doi: 10.1007/s11914-020-00568-5.

Abstract

Purpose of review: To summarize the bone findings, mainly bone mass and fracture risk, in Ehlers-Danlos syndromes (EDS).

Recent findings: Low bone mineral density and fractures seem to be frequent in some of the rare EDS types (kyphoscoliotic, arthrochalasia, spondylodysplastic, and classic-like EDS). For the more prevalent hypermobile and classic EDS types, some case-control studies found mildly decreased bone mineral density, but it was not clear that fracture rates were increased. Nevertheless, abnormalities in vertebral shape seem to be common in classical and hypermobile EDS types. In a cohort of individuals with EDS followed since birth, no fractures were observed during infancy. Bone mineral density varies widely among the different types of EDS, and vertebral abnormalities seem to be common in classical and hypermobile EDS. It might be justified to perform spine radiographs and bone mineral density assessments in newly diagnosed EDS.

Keywords: Bone mass; Bone mineral density; Ehlers–Danlos syndrome; Fracture risk.

Publication types

  • Review

MeSH terms

  • Absorptiometry, Photon
  • Bone Diseases, Metabolic / diagnostic imaging
  • Bone Diseases, Metabolic / epidemiology
  • Ehlers-Danlos Syndrome / epidemiology*
  • Fractures, Bone / epidemiology*
  • Humans
  • Mass Screening
  • Osteoporosis / diagnostic imaging
  • Osteoporosis / epidemiology*
  • Spine / diagnostic imaging

Supplementary concepts

  • Ehlers-Danlos syndrome type 3
  • Ehlers-Danlos syndrome type 6
  • Ehlers-Danlos syndrome, cardiac valvular form