TY - JOUR
T1 - The natural history of children with joint hypermobility syndrome and Ehlers-Danlos hypermobility type
T2 - A longitudinal cohort study
AU - Scheper, Mark C.
AU - Nicholson, Lesley L.
AU - Adams, Roger D.
AU - Tofts, Louise
AU - Pacey, Verity
N1 - Funding Information:
Funding: The study was funded by Arthritis Australia 2015 Zimmer Australia Grant and Arthritis Australia and State and Territory Affiliate Grant, the Arthritis Australia 2013 Barbara Cameron Memorial Grant and the Dutch organization for scientific research (NWO: grant number 023.002.094).
Funding Information:
Disclosure statement: V.P. is an associate investigator on grants from Arthritis Australia and The Menzies Foundation (administered through university institutions) and a Rheumatology Health Professions Association travel scholarship. L.T. received grant funding for this project from Arthritis Australia. All other authors have declared no conflicts of interest.
Publisher Copyright:
© The Author 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved.
PY - 2017/12/1
Y1 - 2017/12/1
N2 - 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≤.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≤.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.
AB - 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≤.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≤.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.
KW - Disability
KW - Ehlers-Danlos (hypermobility type)
KW - Fatigue
KW - Hypermobility syndrome
KW - Multi-systemic dysfunction
KW - Natural course
KW - Pain
UR - http://www.scopus.com/inward/record.url?scp=85038129640&partnerID=8YFLogxK
U2 - 10.1093/rheumatology/kex148
DO - 10.1093/rheumatology/kex148
M3 - Review article
C2 - 28431150
AN - SCOPUS:85038129640
VL - 56
SP - 2073
EP - 2083
JO - British Journal of Rheumatology
JF - British Journal of Rheumatology
SN - 1462-0324
IS - 12
ER -