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?
Abstract
Sir, Joint mobility is a continuous trait that varies with joint location and is strongly influenced by age, gender and ethnic origin. Variation in joint mobility probably begins in utero and can be expressed, often most dramatically, in individuals with heritable syndromes, such as forms of Ehlers–Danlos syndrome (EDS) and various conditions in which joint contracture is a conspicuous feature. In individuals with these disorders, the diagnosis generally depends on the sum of the different features of the condition, in a sense the gestalt of the presentation. In general, the ability to formulate these diagnoses depends in large part on the experience of the clinician and awareness of the classical descriptions of the syndromes. Individuals with syndromic forms of abnormalities in joint mobility tend to fall into the diagnostic realm of the geneticist, paediatrician, rheumatologist or other specialists, depending on the national preferences for referral. Their clinical care is often assumed by rheumatologists and physical therapists. A second and much larger group of individuals occupies the attention of both rheumatologists and geneticists—those who have joint mobility that seems out of the usual range (usually increased) at some point in their lives. They may have acute or chronic subluxation, may have joint-related pain that seems out of proportion to clinical signs and more often than not are female. In part, because of the dissociation of signs and symptoms, this group of individuals with joint hypermobility, tendency to subluxation and joint pain has proved frustrating to clinicians and to the affected individuals themselves and has led to alienation from the medical system and increased demands for recognition and the creation of meaningful, shared and useful formats for diagnosis and treatment.
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Symptomatic as well as asymptomatic joint hypermobility is due to inherited alterations of proteins that lead to laxity of connective tissue, although there is no consensus on the underlying pathophysiology.
“Symptomatic as well as asymptomatic joint hypermobility is due to inherited alterations of proteins that lead to a laxity of connective tissue, although there is no consensus on the underlying pathophysiology (Scheper et al., 2014; Remvig et al., 2011; Remvig, Jensen & Ward, 2007).”
From Prevalence of generalized joint hypermobility, musculoskeletal injuries, and chronic musculoskeletal pain among American university students (2019) · cites this paper as Remvig et al., 2011
The prevalence of GJH in child and adult populations has been reported to range from 2% to almost 65%.
“The prevalence of GJH in child and adult populations has been reported to range from 2% to almost 65% (Scheper et al., 2014; Remvig et al., 2011; Rikken-Bultman, Wellink & Van Dongen, 1997; Simmonds & Keer, 2007; Lamari, Chueire & Cordeiro, 2005; Leone et al., 2009).”
From Prevalence of generalized joint hypermobility, musculoskeletal injuries, and chronic musculoskeletal pain among American university students (2019) · cites this paper as Remvig et al., 2011
Cites
(2000) · PubMed · Cited by 738 · see citing works
Juul-Kristensen, B., Rogind, H., Jensen, D. V., & Remvig, L. (2007) · Rheumatology · doi:10.1093/rheumatology/kem290 · Cited by 299 · see citing works
Self-citationClinical assessment of hypermobility of joints: assembling criteria.
Bulbena, A., Duró, J. C., Porta, M. S., Faus, S., Vallescar, R., & Martín‐Santos, R. (1992) · PubMed · Cited by 199 · see citing works
Self-citationzarate, N., farmer, A. D., grahame, R., mohammed, S. D., knowles, C. H., scott, S. M., & aziz, Q. (2010) · Neurogastroenterology & Motility · doi:10.1111/j.1365-2982.2009.01421.x · Cited by 178 · see citing works
Self-citationHakim, A. J. (2004) · Rheumatology · doi:10.1093/rheumatology/keh279 · Cited by 147 · see citing works
Is Joint Hypermobility Related to Anxiety in a Nonclinical Population Also?
Bulbena, A., Agulló, A., Pailhez, G., Martín-Santos, R., Porta, M., Guitart, J., & Gago, J. (2004) · Psychosomatics · doi:10.1176/appi.psy.45.5.432 · Cited by 76 · see citing works
Self-citationA Novelin VivoSkin Extensibility Test for Joint Hypermobility
Farmer, A. D., DOUTHWAITE, H., Gardiner, S., Aziz, Q., & Grahame, R. (2010) · The Journal of Rheumatology · doi:10.3899/jrheum.091192 · Cited by 25 · see citing works
Self-citationSkin signs in Ehlers–Danlos syndrome: clinical tests and para-clinical methods
Remvig, L., Duhn, P. H., Ullman, S., Arokoski, J. P., Jurvelin, J. S., Safi, A., Jensen, F., Farholt, S., Hove, H. D., & Juul‐Kristensen, B. (2010) · Scandinavian Journal of Rheumatology · doi:10.3109/03009741003781977 · Cited by 22 · see citing works
Self-citationEhlers-Danlos syndromes: Revised nosology, Villefranche, 1997
Beighton, P., Paepe, A. D., Steinmann, B., Tsipouras, P., & Wenstrup, R. J. (1998) · American Journal of Medical Genetics · doi:10.1002/(sici)1096-8628(19980428)77:1
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Juul‐Kristensen, B., Schmedling, K., Rombaut, L., Lund, H., & Engelbert, R. H. H. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31540 · Cited by 229 · see citing works
Self-citationCastori, M. (2012) · ISRN Dermatology · doi:10.5402/2012/751768 · Cited by 222 · see citing works
Engelbert, R. H. H., Juul‐Kristensen, B., Pacey, V., de Wandele, I., Smeenk, S., Woinarosky, N., Sabo, S., Scheper, M. C., Russek, L., & Simmonds, J. V. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31545 · Cited by 177 · see citing works
Self-citationSmith, T. O., Easton, V., Bacon, H., Jerman, E., Armon, K., Poland, F., & Macgregor, A. J. (2014) · Rheumatology · doi:10.1093/rheumatology/ket317 · Cited by 130 · see citing works
Reuter, P. R., & Fichthorn, K. R. (2019) · PeerJ · doi:10.7717/peerj.7625 · Cited by 110 · see citing works
Scheper, M., de Vries, J. E., Verbunt, J., & Engelbert, R. H. H. (2015) · Journal of Pain Research · doi:10.2147/jpr.s64251 · Cited by 105 · see citing works
Self-citationEhlers–Danlos syndrome, hypermobility type: A characterization of the patients' lived experience
Murray, B., Yashar, B. M., Uhlmann, W. R., Clauw, D. J., & Petty, E. M. (2013) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.36293 · Cited by 92 · see citing works
Generalized joint hypermobility in professional dancers: a sign of talent or vulnerability?
Scheper, M. C., de Vries, J. E., de Vos, R., Verbunt, J., Nollet, F., & Engelbert, R. H. H. (2013) · Rheumatology · doi:10.1093/rheumatology/kes220 · Cited by 91 · see citing works
Self-citationClinical heterogeneity in patients with the hypermobility type of Ehlers-Danlos Syndrome
De Wandele, I., Rombaut, L., Malfait, F., De Backer, T., De Paepe, A., & Calders, P. (2013) · Research in Developmental Disabilities · doi:10.1016/j.ridd.2012.11.018 · Cited by 89 · see citing works
Bathen, T., Hångmann, A. B., Hoff, M., Andersen, L. Ø., & Rand‐Hendriksen, S. (2013) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.36060 · Cited by 86 · see citing works
The functional consequences of generalized joint hypermobility: a cross-sectional study
Scheper, M. C., de Vries, J. E., Juul-Kristensen, B., Nollet, F., & Engelbert, R. H. (2014) · BMC Musculoskeletal Disorders · doi:10.1186/1471-2474-15-243 · Cited by 65 · see citing works
Self-citationRemvig, L., Flycht, L., Christensen, K. B., & Juul‐Kristensen, B. (2014) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.36402 · Cited by 66 · see citing works
Self-citationJohannessen, E. C., Reiten, H. S., Løvaas, H., Maeland, S., & Juul-Kristensen, B. (2016) · Disability and Rehabilitation · doi:10.3109/09638288.2015.1102336 · Cited by 61 · see citing works
Self-citationSymptomatic joint hypermobility
Tinkle, B. T. (2020) · Best Practice & Research Clinical Rheumatology · doi:10.1016/j.berh.2020.101508 · Cited by 60 · see citing works
Eccles, J. A., Thompson, B., Themelis, K., Amato, M. L., Stocks, R., Pound, A., Jones, A.-M., Cipinova, Z., Shah-Goodwin, L., Timeyin, J., et al. (2021) · Clinical Medicine · doi:10.7861/clinmed.2020-0743 · Cited by 55 · see citing works
Joint hypermobility syndrome subclassification in paediatrics: a factor analytic approach
Pacey, V., Adams, R. D., Tofts, L., Munns, C. F., & Nicholson, L. L. (2015) · Archives of Disease in Childhood · doi:10.1136/archdischild-2013-305304 · Cited by 40 · see citing works
Castori, M., Dordoni, C., Morlino, S., Sperduti, I., Ritelli, M., Valiante, M., Chiarelli, N., Zanca, A., Celletti, C., Venturini, M., et al. (2015) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31425 · Cited by 40 · see citing works
Schlager, A., Ahlqvist, K., Rasmussen-Barr, E., Bjelland, E. K., Pingel, R., Olsson, C., Nilsson-Wikmar, L., & Kristiansson, P. (2018) · BMC Musculoskeletal Disorders · doi:10.1186/s12891-018-2290-5 · Cited by 35 · see citing works
Joint hypermobility and musculoskeletal injuries in a university-aged population
Reuter, P. R. (2021) · Physical Therapy in Sport · doi:10.1016/j.ptsp.2021.02.009 · Cited by 17 · see citing works
Sanches, S. B., Osório, F. L., Louzada-Junior, P., Moraes, D., Crippa, J. A. S., & Martín-Santos, R. (2014) · Journal of Psychosomatic Research · doi:10.1016/j.jpsychores.2014.08.016 · Cited by 12 · see citing works
Carbone, F., Goelen, N., Fikree, A., Aziz, Q., & Tack, J. (2021) · Neurogastroenterology & Motility · doi:10.1111/nmo.14086 · Cited by 5 · see citing works
Beckers, A. B., Vork, L., Fikree, A., de Ridder, R., Aziz, Q., Masclee, A., & Keszthelyi, D. (2020) · Therapeutic Advances in Gastroenterology · doi:10.1177/1756284820927310 · Cited by 5 · see citing works
- Authors
- Remvig, L., Engelbert, R. H., Berglund, B., Bulbena, A., Byers, P. H., Grahame, R., Juul-Kristensen, B., Lindgren, K.-A., Uitto, J., & Wekre, L. L.
- Affiliations
- Universitat Autònoma de Barcelona (ES)
- Venue
- Rheumatology
- Publisher
- Oxford University Press (OUP)
- Published
- 2011-04-10
- Volume
- 50
- Issue
- 6
- Pages
- 1169-1171
- ISSN
- 1462-0324 (ISSN-L)
- 1462-0332
- OpenAlex
- W2102302711
- Cited by
- 99
- References
- 9
- Citations by year
- 2025: 1 · 2024: 6 · 2023: 3 · 2021: 26 · 2020: 8 · 2019: 6 · 2018: 3 · 2017: 5 · 2016: 8 · 2015: 7 · 2014: 11 · 2013: 8 · 2012: 4
- FWCI
- 4.99
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Lara D. Veeken (publishedVersion)
- PubMed (publishedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- Pure Amsterdam UMC (submittedVersion)
- University of Southern Denmark Research Portal (University of Southern Denmark) (submittedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Health Sciences › Medicine › Pharmacology › Musculoskeletal pain and rehabilitation
- Health Sciences › Health Professions › Pharmacy › Oral and gingival health research
- Keywords
- joint hypermobility, Ehlers-Danlos syndrome hypermobility type, diagnostic criteria, subluxation, joint pain
- MeSH terms
- Adult, Age Factors, Aged, Denmark, Diagnosis, Differential, Ehlers-Danlos Syndrome, Ethnicity, Female, Humans, Joint Instability, Male, Middle Aged, Risk Factors, Severity of Illness Index, Sex Factors, Range of Motion, Articular, Practice Guidelines as Topic, Needs Assessment
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