To (1) establish the association of the most common reported symptoms on disability; and (2) study the effectiveness of treatment on disability in patients with Ehlers-Danlos syndrome–hypermobility type (EDS-HT)/hypermobility syndrome (HMS).
An electronic search (Medical Subject Headings and free-text terms) was conducted in bibliographic databases CENTRAL/MEDLINE.
Comparative, cross-sectional, longitudinal cohort studies and (randomized) controlled trials including patients with HMS/EDS-HT aged ≥17 years were considered for inclusion. A class of symptoms was included when 5 publications were available. In regards to treatment (physical, cognitive interventions), only (randomized) controlled trials were considered. Surgical and medicinal interventions were excluded.
Bias was assessed according to the methodological scoring tools of the Cochrane collaboration. Z-score transformations were applied to classify the extent of disability in comparison with healthy controls and to ensure comparability between studies.
Initially, the electronic search yielded 714 publications, and 21 articles remained for analysis after selection. The following symptoms were included for meta-analysis: pain (n=12), fatigue (n=6), and psychological distress (n=7). Pain (r=.64, P=.021), fatigue (r=.91, P=.011), and psychological distress (r=.86, P=.018) had a significant impact on disability. Regarding treatment, a significant pain reduction was achieved by a variety of physical and cognitive approaches. Treatment effectiveness on disability was not established.
Disability can affect patients with HMS/EDS-HT significantly and is highly correlated with both physical and psychological factors. Although evidence is available that physical and psychological treatment modalities can induce significant pain reduction, the evidence regarding disability reduction is lacking.
List of abbreviations:EDS (Ehlers-Danlos syndrome), EDS-HT (Ehlers-Danlos syndrome–hypermobility type), GJH (generalized joint hypermobility), HDCT (hereditary diseases of connective tissue), HMS (hypermobility syndrome), ICF (International Classification of Functioning, Disability and Health), IQR (interquartile range), MeSH (Medical Subject Headings)
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- Epidemiology of general joint hypermobility and basis for the proposed criteria for benign joint hypermobility syndrome: review of the literature.J Rheumatol. 2007; 34: 804-809
- Joint hypermobility syndrome.BMJ. 2011; 342: c7167
- The hypermobility syndrome. Musculoskeletal complaints associated with generalized joint hypermobility.Ann Rheum Dis. 1967; 26: 419-425
- The revised (Brighton 1998) criteria for the diagnosis of benign joint hypermobility syndrome (BJHS).J Rheumatol. 2000; 27: 1777-1779
- Ehlers-Danlos syndromes: revised nosology, Villefranche, 1997. Ehlers-Danlos National Foundation (USA) and Ehlers-Danlos Support Group (UK).Am J Med Genet. 1998; 77: 31-37
- Joint hypermobility and genetic collagen disorders: are they related?.Arch Dis Child. 1999; 80: 188-191
- The lack of clinical distinction between the hypermobility type of Ehlers-Danlos syndrome and the joint hypermobility syndrome (a.k.a. hypermobility syndrome).Am J Med Genet A. 2009; 149A: 2368-2370
- A tool for clinical reasoning and reflection using the International Classification of Functioning, Disability and Health (ICF) framework and patient management model.Phys Ther. 2011; 91: 416-430
- Need for a consensus on the methods by which to measure joint mobility and the definition of norms for hypermobility that reflect age, gender and ethnic-dependent variation: is revision of criteria for joint hypermobility syndrome and Ehlers-Danlos syndrome hypermobility type indicated?.Rheumatology. 2011; 50: 1169-1171
- Both pain and fatigue are important possible determinants of disability in patients with the Ehlers-Danlos syndrome hypermobility type.Disabil Rehabil. 2011; 33: 706-707
- The Ehlers-Danlos syndrome, a disorder with many faces.Clin Genet. 2012; 82: 1-11
- Children with generalised joint hypermobility and musculoskeletal complaints: state of the art on diagnostics, clinical characteristics, and treatment.Biomed Res Int. 2013; 2013: 121054
- The effectiveness of therapeutic exercise for joint hypermobility syndrome: a systematic review.Physiotherapy. 2014; 100: 220-227
- Generalized joint hypermobility and risk of lower limb joint injury during sport: a systematic review with meta-analysis.Am J Sports Med. 2010; 38: 1487-1497
- Joint hypermobility syndrome: a review for clinicians.J Paediatr Child Health. 2015; 51: 373-380
- Concept analysis of functional status.Int J Nurs Stud. 2004; 41: 457-462
- Using functional status to assess treatment outcomes.Chest. 1994; 106: 1645-1646
- Toward a common language for function, disability, and health.Phys Ther. 2006; 86: 726-734
- The functional consequences of generalized joint hypermobility: a cross-sectional study.BMC Musculoskelet Disord. 2014; 15: 243
- Evaluation of lower limb disability in joint hypermobility syndrome.Rheumatol Int. 2012; 32: 2577-2581
- Impairment and impact of pain in female patients with Ehlers-Danlos syndrome: a comparative study with fibromyalgia and rheumatoid arthritis.Arthritis Rheum. 2011; 63: 1979-1987
- Fatigue in adults with Marfan syndrome, occurrence and associations to pain and other factors.Am J Med Genet A. 2014; 164A: 1931-1939
- A population-based study of demographical variables and ability to perform activities of daily living in adults with osteogenesis imperfecta.Disabil Rehabil. 2010; 32: 579-587
- Medication, surgery, and physiotherapy among patients with the hypermobility type of Ehlers-Danlos syndrome.Arch Phys Med Rehabil. 2011; 92: 1106-1112
- Joint protection and physical rehabilitation of the adult with hypermobility syndrome.Curr Opin Rheumatol. 2011; 23: 131-136
- Hypermobility and the hypermobility syndrome, part 2: assessment and management of hypermobility syndrome: illustrated via case studies.Man Ther. 2008; 13: e1-e11
Higgins J, Green S. Cochrane handbook for systematic reviews of interventions. 2011. Available at: handbook.cochrane.org. Accessed May 8, 2015.
- A systematic review and meta-analysis of whole of community interventions to prevent excessive population weight gain.Prev Med. 2014; 62: 193-200
- The effect of hypermobility on the incidence of injuries in elite-level professional soccer players: a cohort study.Am J Sports Med. 2012; 40: 763-769
- Generalized joint hypermobility in professional dancers: a sign of talent or vulnerability?.Rheumatology. 2013; 52: 651-658
- Is pain the only symptom in patients with benign joint hypermobility syndrome?.Clin Rheumatol. 2015; 34: 1613-1619
- Joint hypermobility syndrome: a common clinical disorder associated with migraine in women.Cephalalgia. 2011; 31: 603-613
- Evaluation of kinesiophobia and its correlations with pain and fatigue in joint hypermobility syndrome/Ehlers-Danlos syndrome hypermobility type.Biomed Res Int. 2013; 2013: 580460
- Static and dynamic body balance following provocation of the visual and vestibular systems in females with and without joint hypermobility syndrome.J Bodyw Mov Ther. 2014; 18: 159-164
- Clinical heterogeneity in patients with the hypermobility type of Ehlers-Danlos syndrome.Res Dev Disabil. 2013; 34: 873-881
- Muscle mass, muscle strength, functional performance, and physical impairment in women with the hypermobility type of Ehlers-Danlos syndrome.Arthritis Care Res (Hoboken). 2012; 64: 1584-1592
- Foot pain and disability in individuals with Ehlers-Danlos syndrome (EDS): impact on daily life activities.Disabil Rehabil. 2005; 27: 164-169
- Chronic pain in patients with the hypermobility type of Ehlers-Danlos syndrome: evidence for generalized hyperalgesia.Clin Rheumatol. 2015; 34: 1121-1129
- Unexpected association between joint hypermobility syndrome/Ehlers-Danlos syndrome hypermobility type and obsessive-compulsive personality disorder.Rheumatol Int. 2014; 34: 631-636
- Generalized joint hypermobility, muscle strength and physical function in healthy adolescents and young adults.Curr Rheumatol Rev. 2015; 10: 117-125
- Improving pain by the stabilization of hyperlax joints.J Orthop Rheumatol. 1996; 9: 46-51
- Multidisciplinary treatment of disability in Ehlers-Danlos syndrome hypermobility type/hypermobility syndrome: a pilot study using a combination of physical and cognitive-behavioral therapy on 12 women.Am J Med Genet A. 2013; 161A: 3005-3011
- Amelioration of symptoms by enhancement of proprioception in patients with joint hypermobility syndrome.Arthritis Rheum. 2004; 50: 3323-3328
- Efficacy of an out-patient pain management programme for people with joint hypermobility syndrome.Clin Rheumatol. 2014; 33: 1665-1669
- Evaluation of knee proprioception and effects of proprioception exercise in patients with benign joint hypermobility syndrome.Rheumatol Int. 2008; 28: 995-1000
- The global burden of hip and knee osteoarthritis: estimates from the Global Burden of Disease 2010 study.Ann Rheum Dis. 2014; 73: 1323-1330
- Multidisciplinary biopsychosocial rehabilitation for chronic low back pain.Cochrane Database Syst Rev. 2014; 9: CD000963
- Burden of disability due to musculoskeletal (MSK) disorders.Best Pract Res Clin Rheumatol. 2014; 28: 353-366
- Lack of consensus on tests and criteria for generalized joint hypermobility, Ehlers-Danlos syndrome: hypermobile type and joint hypermobility syndrome.Am J Med Genet A. 2014; 164A: 591-596
- Joint hypermobility syndrome subclassification in paediatrics: a factor analytic approach.Arch Dis Child. 2015; 100: 8-13
- Factors associated with physical activity in children and adolescents with a physical disability: a systematic review.Dev Med Child Neurol. 2015; 57: 137-148
Published online: March 11, 2016
Supported by the Dutch Society for Scientific Research (NWO), the University of Applied Sciences Amsterdam, Education for Physical Therapy (grant no. 023.002.094).
© 2016 by the American Congress of Rehabilitation Medicine