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Cited by 54Connective tissue disorders researchMusculoskeletal pain and rehabilitationFibromyalgia and Chronic Fatigue Syndrome Research

Diagnosis and Management of Hypermobility Spectrum Disorders in Primary Care

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Atwell, Karina, Michael, William, Dubey, Jared, James, Sarah, Martonffy, Andrea, Anderson, Scott, Rudin, Nathan, Schrager, Sarina·2021·The Journal of the American Board of Family Medicine
DOI 10.3122/jabfm.2021.04.200374
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Abstract

Hypermobility spectrum disorders (HSDs) encompass an array of connective tissue disorders characterized by joint instability and chronic pain. Fatigue and other systemic symptoms that affect daily functioning may occur, as well. Accurate data on incidence and prevalence of HSDs is hampered by lack of awareness of these conditions and the wide heterogeneity of their clinical presentation. Identifying which type of HSD is present is important in guiding appropriate care. In particular, making the diagnosis of hypermobile Ehlers-Danlos syndrome (hEDS) is important, as individuals with hEDS may be at risk for more significant multisystem involvement. Diagnostic criteria for hEDS include measures of joint hypermobility, skin and other connective tissue findings, and lack of evidence of a different type of Ehlers-Danlos syndrome. Beyond accurate diagnosis, HSDs pose many challenges for primary care providers, as ongoing patient education, patient empowerment, and coordination of a multidisciplinary treatment team are integral to proper care. This article describes the incidence and prevalence, pathophysiology, diagnosis, and management of HSDs, including clinical cases exemplifying how joint hypermobility might present within a primary care setting.

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Crossref · 10.3122/jabfm.2021.04.200374

Cites

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Claims the paper makes

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  • Premisecitation

    In college-aged populations, the incidence of hypermobile joints is higher than in older adults.

    “In these college-aged populations, the incidence of hypermobile joints was higher than that in older adults.”
    Quote in the paperNot a substantial claim0 of 2 sources checked
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  • Premisecitation

    A validated patient questionnaire for hypermobility symptoms, with at least two affirmative answers out of five, has 84% sensitivity and 80% specificity for joint hypermobility.

    “On a validated, commonly used patient questionnaire eliciting hypermobility symptoms, an affirmative answer to 2 or more out of 5 questions shows 84% sensitivity and 80% specificity for joint hypermobility (Table 1).”
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    • cites Table 1

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  • Premisecitation

    Kirk and colleagues in 1967 described hypermobility syndrome as familial ligamentous laxity causing recurrent joint pain and periodic joint effusion.

    “Kirk and colleagues first described hypermobility syndrome in 1967, describing a syndrome of familial ligamentous laxity resulting in recurrent joint pain and periodic joint effusion.4”
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  • Premisecitation

    Joint hypermobility is more common in patients diagnosed with anxiety.

    “Joint hypermobility is more common in patients diagnosed with anxiety, 24”
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  • Premisereasoning

    Children of HSD patients may have up to a 50% chance of some degree of hypermobility, given evidence of autosomal dominant inheritance with variable penetrance.

    “Given evidence of an autosomal dominant inheritance pattern with variable penetrance, children of HSD patients may have up to a 50% chance of hypermobility of some degree.”
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    • Rests on an assumption: There is some elevated risk of late-pregnancy back or sacroiliac pain and some risk of early labor in hEDS and probably in HSDs.
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    Premisenone given

    At the same time, type III EDS was renamed hypermobile EDS and given new diagnostic criteria that better distinguish it from G-HSD.

    “At the same time, type III EDS was renamed hypermobile EDS (hEDS) and given a new set of diagnostic criteria that better differentiate it from G-HSD (Table 3).”
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    Premisecitation

    HSDs have a strong genetic basis but no known genetic markers.

    “HSDs have a strong genetic basis but no known genetic markers.”
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    Premisecitation

    The Beighton hypermobility score is commonly used in the assessment of hypermobile patients.

    “The physical examination-based counterpart to this questionnaire is the Beighton hypermobility score (Table 2), which is commonly employed in the assessment of the hypermobile patient.3”
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    • Rests on an assumption: Higher Beighton scores reflect the number of affected joints rather than the degree of joint laxity.

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    Premisecitation

    A recent UK symptom-based survey estimated the prevalence of HSDs in the general population at 3%.

    “A more recent UK symptom-based survey estimated prevalence of HSDs in a general population at 3%. 10”
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    • Rests on an assumption: Accurate incidence and prevalence data for HSDs are hampered by lack of awareness and heterogeneity of clinical presentation.

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    Premisecitation

    The Beighton scoring rubric was originally created for epidemiologic research rather than diagnosis, but it is now used as a measure of generalized hypermobility.

    “Though the Beighton hypermobility scoring rubric was initially introduced as an instrument for epidemiologic research rather than a tool to be used diagnostically, it has now come to be used as a measure of generalized hypermobility. 2”
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    • Rests on an assumption: Higher Beighton scores reflect the number of affected joints rather than the degree of joint laxity.

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    Premisecitation

    In 2017, benign joint hypermobility syndrome was reclassified and renamed hypermobility spectrum disorders, with generalized, peripheral, localized, and historic subtypes.

    “In 2017, JHS was reclassified and renamed the hypermobility spectrum disorders, 5 which are subcategorized into generalized (G-HSD), peripheral (P-HSD), localized (L-HSD), and historic (H-HSD) types.”
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    H-HSD is diagnosed when there is documented prior hypermobility that has disappeared with increasing age.

    “H-HSD is diagnosed in cases of documented prior hypermobility that has disappeared with increased age.7”
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    Premisecitation

    Prevalence of generalized joint hypermobility among college-aged students is between 12.5% and 26%.

    “Prevalence of generalized joint hypermobility among college-aged students ranges from 12.5% to 26%.8,9”
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    • Rests on an assumption: Accurate incidence and prevalence data for HSDs are hampered by lack of awareness and heterogeneity of clinical presentation.

  • Classified as probably not a substantial claim.

    Premisecitation

    HSDs are defined by joint hypermobility on the Beighton score plus one or more secondary musculoskeletal manifestations such as post-traumatic symptoms, pain, altered proprioception, pes planus, kyphosis, scoliosis, or joint misalignment.

    “The HSDs are defined by joint hypermobility using the Beighton score (Table 2) plus 1 or more secondary musculoskeletal manifestations including post-traumatic symptoms, pain, altered proprioception, and a variety of other characteristics like pes planus, kyphosis, scoliosis, or joint misalignment.6”
    Quote in the paperSubstantial claim0 of 1 sources checked
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    • Rests on an assumption: Higher Beighton scores reflect the number of affected joints rather than the degree of joint laxity.

  • May combine two claims.

    Premisecitation

    Factors likely contributing to pain generation and chronicity in HSD include nociceptive pain from structural changes, neuropathic pain, impaired proprioception and muscle weakness, and central sensitization.

    “Factors likely contributing to the generation and chronicity of pain include nociceptive pain due to structural changes in affected joints, muscle, and connective tissue; neuropathic pain; impaired proprioception and muscle weakness; and central sensitization.”
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    • Rests on an assumption: The specific causes and mechanisms of pain in HSD are poorly understood.

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    Premisecitation

    Many consider HSDs and hEDS to be a single clinical entity, with variable manifestations possibly reflecting variability in penetrance.

    “In view of the considerable clinical overlap with HSDs, many consider HSDs and hEDS to be a single clinical entity, with variable manifestations possibly reflecting variability in penetrance.12”
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    Premisecitation

    Pharmacotherapy for HSD/hEDS follows the same principles as for most chronic pain conditions, avoiding opioids and preferring NSAIDs/acetaminophen, topical NSAIDs, lidocaine patches for neuropathic pain, antidepressants (SNRIs or low-dose TCAs), and/or anticonvulsants.

    “The tenets of pharmacotherapy are similar to those in most chronic pain conditions with avoidance of opioids, preference for nonsteroidal anti-inflammatory drugs (NSAIDs)/ acetaminophen, topical NSAIDs, lidocaine patches where neuropathic pain is present, antidepressants (particularly SNRIs or low-dose tricyclic antidepressants [TCAs]), and/or anticonvulsants.31”
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  • Probably not a substantial claim: may not pass S3 (load-bearing). May combine two claims.

    Premisecitation

    Laxity indicates instability, while hypermobility means increased joint range of motion beyond normal.

    “While these 2 terms are often used interchangeably, laxity indicates instability, whereas hypermobility denotes an increase in the joint's range of motion beyond normal.20”
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    Premisecitation

    Core and periarticular strengthening through physical and occupational therapy can enhance proprioception, lower injury risk, and decrease pain while boosting function.

    “Physical and occupational therapy programs centered on strengthening the core and periarticular musculature can improve proprioception, reduce injury, and reduce pain while improving function (see Table 7).”
    Quote in the paperSubstantial claim0 of 1 sources checked
    • cites 10,[33][34][35][36][37][38][39]

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    Premisecitation

    HSD complaints may include autonomic dysfunction and cardiovascular concerns, gastrointestinal issues, anxiety, phobic disorders, major depression, headaches, and chronic fatigue.

    “These complaints may include manifestations of autonomic dysfunction and other cardiovascular concerns, 29 a wide range of gastrointestinal issues, 21 anxiety, phobic disorders, major depression, 25 headaches and chronic fatigue, 10 among others.”
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    • Rests on an assumption: The patient with HSD often has complaints involving multiple organ systems.
    • Rests on an assumption: Many symptoms that bring HSD patients to care are not captured by the validated hypermobility questionnaire.

  • May combine two claims.

    Premisecitation

    Fear of provoking pain and injury may lead to activity avoidance, deconditioning, and exercise intolerance.

    “Fear of provoking pain and injury may lead to avoidance of activity, ultimately resulting in deconditioning and exercise intolerance.”
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    Premisecitation

    Patients with hEDS generally present at a younger age and have more severe symptoms than HSD, and must meet the 2017 international diagnostic criteria.

    “Patients with hEDS generally present to a physician's office at a younger age, have more severe symptoms, and must conform to strict diagnostic criteria put forth in 2017 by the International Consortium on Ehlers-Danlos Syndromes and Related Disorders.3”
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    Premisecitation

    HSD patients tend to have decreased muscle mass and strength, potentially because lax tendons cannot transmit muscle power.

    “In addition, patients tend to have decreased muscle mass and muscle strength, potentially due to increased laxity of tendons, which are unable to transmit power produced by muscles.15,16”
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    Premisecitation

    Most HSD cases are not linked to an identifiable mutation, though pedigree studies show a weak autosomal dominant inheritance pattern with variable penetrance.

    “The vast majority of cases do not seem to be linked to any identifiable mutation, although pedigree studies have shown a weak autosomal dominant inheritance pattern with variable penetrance.10,11”
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    Premisecitation

    HSD patients tend to lack proprioceptive acuity, which may increase their risk of injury.

    “HSD patients tend to lack proprioceptive acuity, which may increase risk of injury.14”
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    Premisecitation

    Deconditioning and exercise intolerance often worsen functional disability and lead to anxiety and depression, which reduce pain tolerance and promote further pain.

    “This often aggravates functional disability and leads to anxiety and depression symptoms, which in turn reduce pain tolerance and promote further pain.16,18”
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    Premisecitation

    Children and young adults with HSD may present with scoliosis, pes planus, genu valgum, genu recurvatum, and/or patellar dislocation or subluxation.

    “Children and young adults may present with scoliosis, pes planus, genu valgum, genu recurvatum, and/or patellar dislocation/subluxation. 10”
    Quote in the paperNot a substantial claim0 of 1 sources checked
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22 of 22

  • The paper relies on this without support.

    Higher Beighton scores reflect the number of affected joints rather than the degree of joint laxity.

    “Higher scores on the Beighton scale simply represent a greater number of affected joints rather than a higher degree of laxity.”

  • The paper relies on this without support.

    The patient with HSD often has complaints involving multiple organ systems.

    “The patient with HSD will often have a range of complaints involving multiple organ systems.”

  • The paper relies on this without support.

    Many symptoms that bring HSD patients to care are not captured by the validated hypermobility questionnaire.

    “Many of these are not included in the validated questionnaire for hypermobility, and these are often the symptoms that draw the attention of patients and cause them to seek care.”

  • The paper relies on this without support.

    Accurate incidence and prevalence data for HSDs are hampered by lack of awareness and heterogeneity of clinical presentation.

    “Accurate data on incidence and prevalence of HSDs is hampered by lack of awareness of these conditions and the wide heterogeneity of their clinical presentation.”

  • The paper relies on this without support.

    The specific causes and mechanisms of pain in HSD are poorly understood.

    “The specific underlying causes and mechanisms of pain in HSD remain poorly understood.”

  • The paper relies on this without support.

    There is some elevated risk of late-pregnancy back or sacroiliac pain and some risk of early labor in hEDS and probably in HSDs.

    “There is some elevated risk of late-pregnancy back or sacroiliac pain, along with some risk of early labor, in hEDS and probably in the HSDs.”

  • The paper relies on this without support.

    HSD and hEDS lie on a single physiologic spectrum, with hEDS representing the more symptomatic end.

    “HSD and hEDS exist on the same physiologic continuum, but hEDS manifests at the more symptomatic edge of the spectrum.”

  • The paper relies on this without support.

    Fatigue and pain are associated, which reduces quality of life.

    “It should also be noted that fatigue and pain seem to be linked, leading to decreased quality of life.”

  • The paper relies on this without support.

    Primary care clinicians commonly encounter patients with hypermobility spectrum disorders.

    “Primary care clinicians will frequently see patients with hypermobility syndrome disorders.”

  • The paper relies on this without support.

    HSD diagnosis is clinical and does not require laboratory or imaging tests for confirmation.

    “While the initial workup for HSD involves eliminating other potential etiologies, the diagnosis is ultimately clinical and requires no laboratory or imaging tests for confirmation.”
10 of 10

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

  • Biomechanical overloading and chronic soft tissue injury likely lead to soft tissue injury manifesting as localized joint arthralgias (nociceptive pain) and, in some, over time, diffuse musculoskeletal pain (central sensitization).

    “This likely leads to soft tissue injury manifesting as localized joint arthralgias (nociceptive pain) and in some, over time, diffuse musculoskeletal pain (central sensitization).[2,8,24,25]”

    From Hypermobility spectrum disorders: A review (2023) · cites this paper as [2]

  • Current theories emphasize localized biomechanical overloading and chronic soft tissue injury due to joint laxity and instability as environmental factors in HSDs.

    “For environmental factors, current theories emphasize localized biomechanical overloading and chronic soft tissue injury due to joint laxity and instability.[2]”

    From Hypermobility spectrum disorders: A review (2023) · cites this paper as [2]

  • Up until 2017, the clinical syndrome of symptomatic joint hypermobility was called joint hypermobility syndrome (JHS) or benign joint hypermobility syndrome (BJHS).

    “Up until 2017 this clinical syndrome was called the joint hyper-mobility syndrome (JHS) or benign joint hypermobility syndrome (BJHS).[2,3]”

    From Hypermobility spectrum disorders: A review (2023) · cites this paper as [2]

  • Studies of college students (typical ages between 18 and 24) report a GJH prevalence between 11%-12.5% and other studies between 12.5%-26%.

    “Studies of “college students” (typical ages between 18 and 24) report a GJH prevalence between 11%-12.5%[16,17] and other between 12.5%-26%.[2,18,19]”

    From Hypermobility spectrum disorders: A review (2023) · cites this paper as [2]

  • Patients with comorbid fibromyalgia may benefit from gabapentin or pregabalin.

    “Patients with comorbid fibromyalgia may benefit from gabapentin or pregabalin.[2]”

    From Hypermobility spectrum disorders: A review (2023) · cites this paper as [2]

Cites

Flagged first, then by citations

1 of the 36 references couldn’t be matched to a known paper.

Research works printed but not linked: 5

  1. Fikree A, Aziz Q, Grahame R. Joint hypermobility syn- drome. Rheum Dis Clin North Am 2013;39:419-30.
  2. Tinkle BT, Levy HP. Symptomatic joint hypermo- bility: the hypermobile type of Ehlers-Danlos syn- drome and the hypermobility spectrum disorders. Med Clin North Am 2019;103:1021-33.
  3. Hauser RA, Phillips HJ. Treatment of joint hypermo- bility syndrome, including Ehlers-Danlos syndrome, with Hackett-Hemwall prolotherapy. Journal of Prolo 2011;3:612-29.
  4. Chopra P, Tinkle B, Hamonet C, et al. Pain man- agement in the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet 2017;175:212-9.
  5. Ferrell WR, Tennant N, Sturrock RD, et al Amelioration of symptoms by enhancement of pro- prioception in patients with joint hypermobility syndromes. Arthritis Rheumatism 2004;50:3323-8. 848 JABFM July-August 2021 Vol. 34 No. 4 http://www.jabfm.org on 20 August 2023 by guest. Protected by copyright. http://www.jabfm.org/ J Am Board Fam Med: first published as 10.3122/jabfm.2021.04.200374 on 26 July 2021. Downloaded from

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Authors
Atwell, K., Michael, W., Dubey, J., James, S., Martonffy, A., Anderson, S., Rudin, N., & Schrager, S.
Affiliations
University of Wisconsin–Madison (US)
ORCID
Venue
The Journal of the American Board of Family Medicine
Publisher
American Board of Family Medicine (ABFM)
Published
2021-07
Volume
34
Issue
4
Pages
838-848
ISSN
  • 1557-2625 (ISSN-L)
  • 1558-7118
DOI
10.3122/jabfm.2021.04.200374
OpenAlex
W3184166575
Cited by
52
References
36
Citations by year
2026: 9 · 2025: 10 · 2024: 13 · 2023: 11 · 2022: 6 · 2021: 3
FWCI
1.22
Type
journal article
Language
en
Open access
Locations
  • The Journal of the American Board of Family Medicine (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
  • Health Sciences › Medicine › Pharmacology › Musculoskeletal pain and rehabilitation
  • Health Sciences › Medicine › Psychiatry and Mental health › Fibromyalgia and Chronic Fatigue Syndrome Research
Keywords
hypermobility spectrum disorders, hypermobile Ehlers-Danlos syndrome, connective tissue diseases, joint hypermobility, chronic pain, fatigue, diagnosis, primary care management, patient education, multidisciplinary care, incidence and prevalence, pathophysiology
MeSH terms
Diagnosis, Differential, Humans, Joint Instability, Primary Health Care

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