The natural history of children with joint hypermobility syndrome and Ehlers–Danlos hypermobility type: a longitudinal cohort study

Mark C. Scheper, Leslie Nicholson, Roger Adams, Louise Tofts, Verity Pacey

Research output: Contribution to journalArticlepeer-review

21 Citations (Scopus)

Abstract

Objectives: The objective of the manuscript was to describe the natural history of complaints and disability in children diagnosed with joint hypermobility syndrome (JHS)/Ehlers–Danlos-hypermobility type (EDS-HT) and to identify the constructs that underlie functional decline.
Methods: One hundred and one JHS/EDS-HT children were observed over 3 years and assessed at three time points on the following: functional impairments, quality of life, connective tissue laxity, muscle function, postural control and musculoskeletal and multi-systemic complaints. Cluster analysis was performed to identify subgroups in severity. Clinical profiles were determined for these subgroups, and differences were assessed by multivariate analysis of covariance. Mixed linear regression models were used to determine the subsequent trajectories. Finally, an exploratory factor analysis was used to uncover the underlying constructs of functional impairment.
Results: Three clusters of children were identified in terms of functional impairment: mild, moderately and severely affected. Functional impairment at baseline was predictive of worsening trajectories in terms of reduced walking distance and decreased quality of life (P ⩽ 0.05) over 3 years. Multiple interactions between the secondary outcomes were observed, with four underlying constructs identified. All four constructs (multi-systemic effects, pain, fatigue and loss of postural control) contributed significantly to disability (P ⩽ 0.046).
Conclusion: Children diagnosed with JHS/EDS-HT who have a high incidence of multi-systemic complaints (particularly, orthostatic intolerance, urinary incontinence and diarrhoea) and poor postural control in addition to high levels of pain and fatigue at baseline are most likely to have a deteriorating trajectory of functional impairment and, accordingly, warrant clinical prioritization.
Original languageEnglish
Pages (from-to)2073–2083
Number of pages11
JournalRheumatology (United Kingdom)
Volume56
Issue number12
Early online date17 Apr 2017
DOIs
Publication statusPublished - 1 Dec 2017

Keywords

  • hypermobility syndrome
  • Ehlers-Danlos (hypermobility type)
  • disability
  • natural course
  • pain
  • fatigue
  • multi-systemic dysfunction
  • Multi-systemic dysfunction
  • Hypermobility syndrome
  • Fatigue
  • Disability
  • Pain
  • Natural course

Fingerprint Dive into the research topics of 'The natural history of children with joint hypermobility syndrome and Ehlers–Danlos hypermobility type: a longitudinal cohort study'. Together they form a unique fingerprint.

Cite this