Joint hypermobility

doi:10.1016/j.berh.2003.08.001

Best Practice & Research Clinical Rheumatology

Volume 17, Issue 6, December 2003, Pages 989-1004

https://doi.org/10.1016/j.berh.2003.08.001 Get rights and content

Abstract

Joint hypermobility is an area of neglect in rheumatology. That is not to say it is overlooked by rheumatologists. It is spotted when sought, but for many unfortunate patients, here the story ends. The act of recognition becomes the goal in itself rather than the medium through which effective therapy can be provided. This chapter serves to reinforce the clinical and epidemiological importance of a common disorder whose significance is under-appreciated and impact largely ignored. In contradistinction to our earlier chapter, published in 2000, which took for its remit the heritable disorders of connective tissue in general, the current one focuses on the commonly encountered (so-called benign) joint hypermobility syndrome, its recognition, epidemiology, clinical features and management according to the most recent literature.

Section snippets

The prevalence of hypermobility and the hypermobility syndrome

It is important to distinguish hypermobility, which describes the often asymptomatic increased range of joint or spinal movement, from hypermobility syndrome, its symptomatic counterpart. Population studies demonstrate a wide variation in the prevalence of joint hypermobility; its presence is influenced by age, gender and ethnicity. Hypermobility diminishes with age from childhood onward, is about three times more common in females than males and is seen more often in Asian and African races

Hypermobility as an asset

Greater agility provided by joint laxity may favour performing artists, where the prevalence of hypermobility is significantly higher than that of the general population. It appears that hypermobility acts as a positive selection factor for entry into ballet for both girls and boys. Polyarticular hypermobility is seen in this group as well as in adult dancers in as many as 90%.¹⁶ Hypermobile violinists, flautists and pianists (of all ages) with lax finger and/or wrist joints suffer less pain

Identifying hypermobility

The conventional method of identifying hypermobility, irrespective of cause, is to apply the Beighton nine-point scoring system established in 1973. This requires the performance of number of manoeuvres listed in Table 1. Conventionally, an arbitrary number of four or more out of nine is taken to be diagnostic of ‘generalized’ hypermobility.

This Beighton nine-point score is widely used as a clinical screening test for hypermobility. However, it cannot be relied upon to identify pauciarticular

What is the JHS?

Many authorities nowadays believe that JHS, as manifest in patients seeking medical help, is almost certainly identical with the EDS hypermobility type (formerly EDS type III).¹ This question, however, is controversial and will be satisfactorily resolved only when the precise genetic basis of both conditions has been established. The term ‘benign’ denotes a normal life expectancy and, in effect, the absence of cardiac and arterial disorders, a lesser tendency towards osteoporosis and milder

Presenting symptoms in the JHS

JHS patients may present with a wide variety of musculoskeletal symptoms encompassing spine, joint, ligament, muscle, tendon and bone, and at first sight, appear no different from other rheumatology patients. What set JHS patients apart are the unusual frequency, range and number of lesions at any one time or over the life span of the patient. The most common symptom is pain. This ranges from localized (often recurrent) acute pain, perhaps a consequence of tissue injury or overuse, to chronic

ManagEment

The approach recommended here is the one adopted in the hypermobility clinics established by the authors at the University College London Hospitals and at Whipps Cross University Hospital. The approach to management is of necessity a multidisciplinary one but remaining holistic in its outlook. Each patient with JHS will have their own individual set of clinical problems and will require an individualized treatment plan tailored to particular needs.

Our strategy is to

1.
treat the treatable

Summary

This chapter draws readers' attention to a poorly understood backwater of rheumatology, the HDCTs, and, in particular, to a common, debilitating but under-recognized condition, the JHS. Hopefully, it will enable rheumatologists and others to familiarize themselves with its not inconsiderable significance, the nature of its variegated clinical manifestations, its prognosis and its effective treatment.

References (52)

R Grahame
Heritable disorders of connective tissue
Best Practice and Research Clinical Rheumatology
(2000)
A Sacheti et al.
Chronic pain is a manifestation of the Ehlers–Danlos syndrome
Journal of Pain and Symptom Management
(1997)
Y Gazit et al.
Dysautonomia in the hypermobility syndrome
American Journal of Medicine
(2003)
P.A Norton et al.
Genitourinary prolapse and joint hypermobility in women
Obstetrics and Gynecology
(1995)
C.W Schauberger et al.
Peripheral joint laxity increases in pregnancy but does not correlate with serum relaxin levels
American Journal of Obstetrics and Gynecology
(1996)
P.W.J van Dongen et al.
Hypermobility and peripartum pelvic pain syndrome in pregnant South African women
European Journal of Obstetrics, Gynecology and Reproductive Biology
(1999)
P.C Rowe et al.
Orthostatic intolerance and chronic fatigue syndrome associated with Ehlers–Danlos syndrome
Journal of Pediatrics
(1999)
A Bulbena et al.
Anxiety disorders in the joint hypermobility syndrome
Psychiatry Research
(1993)
D.F Barron et al.
Joint hypermobility is more common in children with chronic fatigue syndrome than in healthy controls
Journal of Pediatrics
(2002)
M Holzberg et al.
The Ehlers–Danlos syndrome: recognition, characterization, and importance of a milder variant of the classic form. A preliminary study
Journal of the American Academy of Dermatology
(1988)

L.C Decoster et al.

Prevalence and features of joint hypermobility among adolescent athletes

Archives of Pediatrics and Adolescent Medicine

(1997)

Cited by (259)

Beyond Confirmed Mast Cell Activation Syndrome: Approaching Patients With Dysautonomia and Related Conditions
2024, Journal of Allergy and Clinical Immunology: In Practice
Allergist-immunologists face significant challenges as experts in an ever-evolving field of neuroimmunology. Among these challenges is the increasingly frequent need to counsel patients with suspected mast cell activation disorders about perceived comorbidities, which may include hypermobile Ehlers-Danlos syndrome, amplified pain syndrome, fibromyalgia, burning sensation syndromes, migraines, irritable bowel syndrome, and postural orthostatic tachycardia syndrome. Patients may experience comorbid anxiety, panic disorder, and depression associated with disturbed sleep, fatigue, and cognitive impairment that often worsen when their physical symptoms increase in severity. These conditions may mimic mast cell activation disorders and are emotionally taxing for patients and clinicians because they are often accompanied by vague diagnostic courses, perceived unmanageability, social stigma, and significant impairment in quality of life. Combined with relatively poorly researched therapies, it is no surprise that clinicians may feel overwhelmed or find it difficult to provide consistently compassionate care for this population. In this article, we review available therapies for these conditions, which run the gamut from physical therapy to antidepressants to multimodal pain control. We highlight the benefit of multidisciplinary care within the primary care home, which includes an important role by the allergist-immunologist. By outlining simple approaches to initial treatment, we hope to empower clinicians with the tools needed to curb emotional burnout and embrace this patient population with compassion.
Folate-dependent hypermobility syndrome: A proposed mechanism and diagnosis
2023, Heliyon
Hypermobility involves excessive flexibility and systemic manifestations of connective tissue fragility. We propose a folate-dependent hypermobility syndrome model based on clinical observations, and through a review of existing literature, we raise the possibility that hypermobility presentation may be dependent on folate status. In our model, decreased methylenetetrahydrofolate reductase (MTHFR) activity disrupts the regulation of the ECM-specific proteinase matrix metalloproteinase 2 (MMP-2), leading to high levels of MMP-2 and elevated MMP-2-mediated cleavage of the proteoglycan decorin. Cleavage of decorin leads ultimately to extracellular matrix (ECM) disorganization and increased fibrosis. This review aims to describe relationships between folate metabolism and key proteins in the ECM that can further explain the signs and symptoms associated with hypermobility, along with possible treatment with 5-methyltetrahydrofolate supplementation.
Biomechanical and bioelasticity analysis of the hypermobile knee
2023, Cartilage Tissue and Knee Joint Biomechanics: Fundamentals, Characterization and Modelling
To fully understand knee joint biomechanics, it is essential to understand the biomechanics of the hypermobile knee. The average normal range of motion of the knee joint at the sagittal plane is 0–140 degrees of knee extension-flexion. However, a change in the knee angle from zero extension to ten degrees or more, beyond the normative full extension angle, would have a substantial impact on the biomechanics of the knee joint. First, in this chapter, essential definitions of hypermobility as a clinical feature and hypermobility-related disorders are explained, and the prevalence figures are presented to highlight the widespread nature of the hypermobile knee. Next, essential biomechanical findings determined using three-dimensional motion analysis systems are highlighted, and the bioelasticity of the knee joint stabilization structures explored using sonoelastography ultrasound-based systems is discussed. Overall, this chapter provides a clear understanding of the biomechanics and the bioelasticity of the knee stabilization system in the hypermobile knee.
No Clinical or Radiographic Difference Seen in Arthroscopic Bankart Repair With Knotted Versus Knotless Suture Anchors: A Randomized Controlled Trial at Short-Term Follow-Up
2022, Arthroscopy - Journal of Arthroscopic and Related Surgery
The objective of this study was to compare the clinical and radiographic results of patients undergoing arthroscopic repair of labral lesions using knotted and knotless anchors.
Sixty-four patients with anterior labral lesions (bone defects of the glenoid up to 13.5% or up to 20% if the instability severity index score was less than 4) and without other shoulder pathologies were randomly assigned to 2 groups, with 32 patients in each group. Clinical outcomes were assessed 6, 12, and 24 months after surgery by the Rowe score, Western Ontario shoulder instability index (WOSI), single assessment numeric evaluation (SANE), visual analog scale for pain (VASp), range of motion, and rate of postoperative recurrence. Postoperative magnetic resonance imaging (MRI) was performed, and the anterior and inferior labrum glenoid height indexes and anterior and inferior labral slopes were measured. The primary endpoint was the Rowe score at 24 months postoperatively.
Fifty-one patients, 24 in the knotted group and 27 in the knotless group, completed 24 months of follow-up. At 24 months, the Rowe scores were 81.7 ± 19.9 points and 85.9 ± 14 points, respectively (P = .623); the WOSI scores were 509.2 ± 480.1 points and 555.9 ± 393.6 points, respectively (P = .533); the SANE scores were 90.7 ± 18.7 points and 89.2 ± 14.8 points, respectively (P = .427); and the VASp scores were 1.7 ± 2.5 points and 2.5 ± 2.7 points, respectively (P = .275). There was no significant difference in range of motion, postoperative recurrence, or MRI parameters between the groups. All subjects (100%) in both groups exceeded the minimal clinically important difference of 9.7 for the Rowe score (P > .999).
Repair of Bankart's lesion through the use of knotted and knotless suture anchors yielded similar clinical and radiographic results on analysis at 24 months after operation.
Level I. Randomized controlled clinical trial.
Arthroscopic Capsular Plication for Multidirectional Shoulder Instability in Hypermobile Ehlers-Danlos Syndrome Patients
2021, Arthroscopy Techniques
Patients with hypermobile Ehlers-Danlos syndrome commonly present with painful shoulder disorders mostly represented by a multidirectional instability and with often painless episode of atraumatic shoulder dislocation and/or subluxation. Because of the generalized soft tissue fragility, most of the surgical treatments of shoulder instability, as the Bankart repair or the Latarjet procedure becomes not effective, exposing a risk of failure. Then, capsular plication has to be considered in order to reduce the articular joint volume and to diminish the mobility of the joint. Two main benefits of this surgical procedure are that it doesn’t modify the anatomy or damage the labrum or cartilage that is healthy. The purpose of this Technical Note is to present the arthroscopic capsular plication for the treatment of patient with hypermobile Ehlers-Danlos syndrome, which is associated with a multidirectional shoulder instability.
Echocardiographic abnormalities and joint hypermobility in Chinese patients with Osteogenesis imperfecta
2024, Orphanet Journal of Rare Diseases

View all citing articles on Scopus

View full text

6Joint hypermobility

Abstract

Section snippets

The prevalence of hypermobility and the hypermobility syndrome

Hypermobility as an asset

Identifying hypermobility

What is the JHS?

Presenting symptoms in the JHS

ManagEment

Summary

Best Practice and Research Clinical Rheumatology

Journal of Pain and Symptom Management

American Journal of Medicine

Obstetrics and Gynecology

American Journal of Obstetrics and Gynecology

European Journal of Obstetrics, Gynecology and Reproductive Biology

Journal of Pediatrics

Psychiatry Research

Journal of Pediatrics

Journal of the American Academy of Dermatology